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Oxford Medical Publications

Oxford Handbook of

General Practice

Published and forthcoming Oxford Handbooks Oxford Handbook for the Foundation Programme 3e Oxford Handbook of Acute Medicine 3e Oxford Handbook of Anaesthesia 3e Oxford Handbook of Applied Dental Sciences Oxford Handbook of Cardiology 2e Oxford Handbook of Clinical and Laboratory Investigation 3e Oxford Handbook of Clinical Dentistry 5e Oxford Handbook of Clinical Diagnosis 2e Oxford Handbook of Clinical Examination and Practical Skills Oxford Handbook of Clinical Haematology 3e Oxford Handbook of Clinical Immunology and Allergy 3e Oxford Handbook of Clinical Medicine – Mini Edition 8e Oxford Handbook of Clinical Medicine 8e Oxford Handbook of Clinical Pathology Oxford Handbook of Clinical Pharmacy 2e Oxford Handbook of Clinical Rehabilitation 2e Oxford Handbook of Clinical Specialties 9e Oxford Handbook of Clinical Surgery 4e Oxford Handbook of Complementary Medicine Oxford Handbook of Critical Care 3e Oxford Handbook of Dental Patient Care 2e Oxford Handbook of Dialysis 3e Oxford Handbook of Emergency Medicine 4e Oxford Handbook of Endocrinology and Diabetes 2e Oxford Handbook of ENT and Head and Neck Surgery Oxford Handbook of Epidemiology for Clinicians Oxford Handbook of Expedition and Wilderness Medicine Oxford Handbook of Gastroenterology & Hepatology 2e Oxford Handbook of General Practice 4e Oxford Handbook of Genetics Oxford Handbook of Genitourinary Medicine, HIV and AIDS 2e Oxford Handbook of Geriatric Medicine Oxford Handbook of Infectious Diseases and Microbiology Oxford Handbook of Key Clinical Evidence Oxford Handbook of Medical Dermatology Oxford Handbook of Medical Imaging Oxford Handbook of Medical Sciences 2e Oxford Handbook of Medical Statistics Oxford Handbook of Nephrology and Hypertension Oxford Handbook of Neurology Oxford Handbook of Nutrition and Dietetics 2e Oxford Handbook of Obstetrics and Gynaecology 2e Oxford Handbook of Occupational Health 2e Oxford Handbook of Oncology 3e Oxford Handbook of Ophthalmology 2e Oxford Handbook of Oral and Maxillofacial Surgery Oxford Handbook of Paediatrics 2e Oxford Handbook of Pain Management Oxford Handbook of Palliative Care 2e Oxford Handbook of Practical Drug Therapy 2e Oxford Handbook of Pre-Hospital Care Oxford Handbook of Psychiatry 3e Oxford Handbook of Public Health Practice 2e Oxford Handbook of Reproductive Medicine & Family Planning Oxford Handbook of Respiratory Medicine 2e Oxford Handbook of Rheumatology 3e Oxford Handbook of Sport and Exercise Medicine 2e Oxford Handbook of Tropical Medicine 3e Oxford Handbook of Urology 3e

Oxford Handbook of

General Practice Fourth Edition

Chantal Simon General Practitioner and Executive Editor, InnovAiT

Hazel Everitt General Practitioner and Clinical Lecturer, University of Southampton

Françoise van Dorp General Practitioner

Guest author

Matt Burkes General Practitioner

1

3 Great Clarendon Street, Oxford, OX2 6DP, United Kingdom Oxford University Press is a department of the University of Oxford. It furthers the University’s objective of excellence in research, scholarship, and education by publishing worldwide. Oxford is a registered trade mark of Oxford University Press in the UK and in certain other countries © Oxford University Press2014 The moral rights of the authors have been asserted First Edition published in 2002 Second Edition publishedin2005 Third Edition publishedin2010 Fourth Edition publishedin2014 Impression:1 All rights reserved. No part of this publication may be reproduced, stored in a retrieval system, or transmitted, in any form or by any means, without the prior permission in writing of Oxford University Press, or as expressly permitted by law, by licence or under terms agreed with the appropriate reprographics rights organization. Enquiries concerning reproduction outside the scope of the above should be sent to the Rights Department, Oxford University Press, at the addressabove You must not circulate this work in any other form and you must impose this same condition on any acquirer Published in the United States of America by Oxford University Press 198 Madison Avenue, New York, NY 10016, United States of America British Library Cataloguing in PublicationData Data available Library of Congress Control Number: 2013945300 ISBN 978–0–19–967103–8 Printed in China by C&C Offset Printing Co. Ltd. Oxford University Press makes no representation, express or implied, that the drug dosages in this book are correct. Readers must therefore always check the product information and clinical procedures with the most up-to-date published product information and data sheets provided by the manufacturers and the most recent codes of conduct and safety regulations. The authors and the publishers do not accept responsibility or legal liability for any errors in the text or for the misuse or misapplication of material in this work. Except where otherwise stated, drug dosages and recommendations are for the non-pregnant adult who is not breast-feeding Links to third party websites are provided by Oxford in good faith and for information only. Oxford disclaims any responsibility for the materials contained in any third party website referenced in thiswork

v

Contents

Foreword╇ vii Acknowledgements╇ ix Abbreviations used in the text╇ xi 1 What is general practice? 2 Contracts 3 Practice management 4 Education, monitoring, and research 5 Consulting and certification 6 Medicines and prescribing 7 Minor surgery 8 Healthy living 9 Chronic disease and elderly care 10 Cardiology and vascular disease 11 Respiratory medicine 12 Endocrinology 13 Gastrointestinal medicine 14 Renal medicine and urology 15 Musculoskeletal problems 16 Neurology 17 Dermatology 18 Infectious disease 19 Haematology and immunology 20 Breast disease 21 Gynaecology

╇╇1 ╇17 ╇33 ╇59 ╇87 135 157 169 197

231 293 341 373 435 471 533 587 643 659 687 701

vi

contents

22 Sexual health and contraception ╇ 733 23 Pregnancy ╇781 24 Child health ╇ 845 25 Ear, nose, and throat ╇ 929 26 Ophthalmology ╇953 27 Mental health ╇ 985 28 Cancer and palliative care 1019 29 Emergencies in general practice 1049 Index╇ 1129

vii

Foreword The pace of change in general practice seems to be accelerating, and there has been a lot of change for the fourth edition of the Oxford Handbook of General Practice. For this new edition, we welcome Dr Matt Burkes as Guest Editor. He has recently finished GP training and has been responsible for the chapters on mental health and skin problems. Since the last edition of the OHGP, clinical general practice has moved on apace with new treatments, guidelines, and care pathways, changing the way in which we work. There has been a continued move of work from secondary care to primary care to enable patients to be cared for closer to home, and our ageing population means that many of our patients have multiple morbidities that must be managed simultaneously. Furthermore, GPs must continue to provide high-quality care within an environment of increasing public expectation and decreasing financial resources. The administration of general practice in the UK is also changing, as the devolved nations’ healthcare systems move further apart. GP practices in the UK now need to meet standards imposed by the Care Quality Commission, and perhaps the biggest change is the abolition of Primary Care Trusts (just in England) and their replacement with GP-led commissioning through Clinical Commissioning Groups. Revalidation has also become a reality. All GPs in the UK registered with the General Medical Council must demonstrate their fitness to practise every five years. Perhaps unsurprisingly, GP training is changing too. General practice has traditionally had the shortest training of all specialties in the UK. The need for a widening clinical skills and knowledge base, improved generalist skills to promote health, prevent disease and manage people with complex problems in a variety of different settings, and improved public health and leadership skills have led to a successful bid by the RCGP to enhance and lengthen GP training to prepare GPs fit for the future. To address all these factors, within the non-clinical sections of this new edition of the OHGP, we have included new sections on revalidation, enhanced and extended GP training, commissioning, changes to benefits for low income, sickness and disability, and Care Quality Commission registration. In addition, as well as updating all the clinical information in line with current guidance within the clinical sections of this edition, we have included photographs within the text for the first time. Due to space limitations, we have used these initially just within the dermatology and ophthalmology chapters, and we hope that you will find them useful. As always, we welcome any feedback from our readers. We would also like to thank the many of you who have contacted us to point out errors, omissions, and ways to improve the OHGP in the past. It is thanks to you and the feedback that you provide that the OHGP continues to develop to meet your day-to-day needs. CS HE FvD

ix

Acknowledgements This book would not have come into being without input from a large number of individuals. First, we would like to thank our very select and helpful editorial team at Oxford University Press: Helen Liepman, Liz Reeves, and Michael Hawkes. Helen was brave enough to commission us to write the first edition of the Oxford Handbook of General Practice (OHGP) in 1999 and then moved to other projects. Liz left us midway through preparation of the third edition of the OHGP to go on maternity leave. Therefore, it has been lovely to have both back on the team for this fourth edition. Next, we would like to thank our long-suffering families. Writing and editing a book like the OHGP is very time-consuming, and we all have other ‘day jobs’. Without the support of our families, a project like the OHGP would not be possible. In alphabetical order, we would like to give special thanks to our spouses: David Gough, Ruth Searle, Ian Wright, and Peter Wynn—and children: Adam, Ben, Charlie, Emma, Emily, Flint, Hannah, Helena, Kate, Leif, River, and Sophie. The OHGP has always had a ‘hands-on’ feel. That is because it is written by practising GPs who learn new things every day from their patients and colleagues. We would like to thank all those that we work with for their help and support. In particular, we would like to thank the authors, reviwers, and entire Editorial Board of InnovAiT (the RCGP Journal for GPs in training), the staff of the Department of Primary Medical Care at the University of Southampton and the GPs, patients, and staff of the following practices: • The Banks and Bearwood Medical Centres, Bournemouth, Dorset • Dr Godfrey and Partners, Totton, Hampshire • Wilton Health Centre, Wilton, Wiltshire • The Orchard Partnership, Wiltshire • Langley House Surgery, Chichester, West Sussex • Maywood Surgery, Bognor Regis, West Sussex Finally, we would like to give special thanks to all the other many individuals who contributed, either directly or indirectly, to this edition of the OHGP. We do not always know who you are, as much of the OHGP is peer-reviewed anonymously, but your expert input has been extremely valuable. In particular, we would like to thank: • Mr Richard Newsom (Consultant Ophthalmologist, Southampton University Hospitals Trust) for his expert help co-authoring Chapter 26 (Ophthalmology). • Dr Helen Dignum (Consultant Haematologist, Portsmouth Hospitals Trust) for her help providing expert review of Chapter 19 (Haematology and immunology) and answering the tricky questions that came up when that chapter was sent for anonymous peer review. • Dr Alice Shiner (GP) and Mr Nikolaos Burbos (Consultant Gynaecologist, Norfolk and Norwich University Hospitals Trust) for their expert review of Chapter 21 (Gynaecology).

x Acknowledgements • Dr Max Watson (Consultant in Palliative Medicine, Northern Ireland Hospice) for his help with contributions to and review of Chapter 28 (Cancer and palliative care). • Dr Roger Gadsby (GP and Associate Clinical Professor, University of Warwick) for his review and expert feedback on Chapter 12 (Endocrinology). • Dr Ben Riley (GP and Medical Director Curriculum, RCGP) for his help providing information about GP training and the RCGP Curriculum for Chapter 4 (Education, monitoring, and research). • Dr Waseem Aslam for his helpful input for the section on multicultural medicine in Chapter 5. • RCGP ‘Supporting Carers in General Practice’ project (Clinical Leads, Dr Elizabeth Muir, Dr Chantal Simon and Mrs Judith Cameron, the RCGP Professional Programmes Team, and Dr Moira Fraser of the Carers Trust) for providing the information for the pages about carers in Chapter 9. • The Cornwall Partnership CASC Team (Dr Rohit Shankar, Dr Giles Richards, Dr Richard Laugharne, and Dr Karl Rathbone) for allowing us to ask as many questions as we liked about mental health for Chapter 27. • Previous authors and editors of the OHGP Dr Brian Stevenson, Dr Jon Birtwistle, Dr Knut Schroeder, and Professor Tony Kendrick. If there any omissions from this list, we apologize. Please tell us, and we will add your name to the list at the first opportunity. CS HE FvD MB

xi

Abbreviations used in the text Evidence-based superscripts N

NICE guideline

G

Guideline from a major guideline-producing body

C

Cochrane review

CE

Clinical evidence

S

Systematic review or meta-analysis published in a major peer-reviewed journal

R

Randomized controlled trial published in a major peer-reviewed journal

Referral times E U

Emergency admission

S R

Soon referral

Urgent referral Routine referral

Handbook symbols 0 • b M F 5 4 X

Note

p s i d l 7 8 9

Primary

Warning OHGP cross reference Weblink Telephone number Female Male Controversy Secondary Increased/increasing Decreased/decreasing Leading to/resulting in Approximately Approximately equal With or without

xii Abbreviations used in the text Standard abbreviations AA

Attendance Allowance

AAA

abdominal aortic aneurysm

ABPI

ankle-brachial pressure index

ABPM

ambulatory blood pressure monitoring

ACE

angiotensin-converting enzyme

ACS

acute coronary syndrome

ACTH

adrenocortocotrophic hormone

ADH

antidiuretic hormone

ADHD

attention deficit hyperactivity disorder

AED

automated external defibrillator

AF

atrial fibrillation

AFP/AFP

alpha fetoprotein

AIDS

acquired immune deficiency syndrome

Alk phos

alkaline phosphatase

ALT

alanine aminotransferase

ANF

antinuclear factor

APH

antepartum haemorrhage

ARB

angiotensin receptor blocker

ASD

atrial septal defect

ASO

antistreptolysin O

AST

aspartate aminotransferase

AV

arterio-venous

AXR

abdominal X-ray

BASHH

British Association for Sexual Health and HIV

BCG

Bacille Calmette–Guérin

bd

twice daily

BHCG

beta-human chorionic gonadotrophin

BMA

British Medical Association

BMJ

British Medical Journal

BNF

British National Formulary

BP

blood pressure

bpm

beats per minute

Abbreviations used in the text

Ca2+

calcium

CABG

coronary artery bypass graft

CCF

congestive cardiac failure

CCG

Clinical Commissioning Group

CHC

combined hormonal contraception

CHD

coronary heart disease

CNS

central nervous system

COC

combined oral contraceptive

COPD

chronic obstructive airways disease

Cr

creatinine

CRP

C-reactive protein

CT

computerized tomography

CVA

cerebrovascular accident

CVD

cardiovascular disease

CVS

cardiovascular system

CXR

chest X-ray

d

days

DDH

developmental dysplasia of the hip

DES

Directed Enhanced Service

DH

Department of Health

DIPJ

distal interphalangeal joint

DLA

Disability Living Allowance

DM

diabetes mellitus

DN

district nurse

DRE

digital rectal examination

DVLA

Driving and Vehicle Licensing Authority

DVT

deep vein thrombosis

EBV

Epstein–Barr virus

ECG

electrocardiogram

Echo

echocardiogram

EEG

electroencephalogram

ENT

ear, nose, and throat

EPAU

early pregnancy assessment unit

xiii

xiv Abbreviations used in the text ESA

Employment and Support Allowance

ESR

erythrocyte sedimentation rate

ESRD

end-stage renal disease

FBC

full blood count

FBG

fasting blood glucose

FEV1

forced expiratory volume in 1 second

FH

family history

FSH

follicle-stimulating hormone

FSRH

Faculty of Sexual and Reproductive Healthcare

FVC

forced vital capacity

g

grams

GA

general anaesthetic

GI

routine referral

GGT

gamma glutamyl transferase

GMC

General Medical Council

GMS

General Medical Services

GP

general practitioner

GPC

General Practitioner Committee

GTN

glyceryl trinitrate

GTT

glucose tolerance test

GU

genito-urinary

GUM

genito-urinary medicine

h

hours

Hb

haemoglobin

HbA1c

glycosylated haemoglobin

HBPM

home blood pressure monitoring

HBsAg

hepatitis B surface antigen

HDL

high density lipoprotein

Hib

Haemophilus influenzae type b

HIV

human immunodeficiency virus

HOCM

hypertrophic obstructive cardiomyopathy

HRT

hormone replacement therapy

Abbreviations used in the text

HSV

herpes simplex virus

HV

health visitor

HVS

high vagin*l swab

ICP

intracranial pressure

Ig

immunoglobulin

IHD

ischaemic heart disease

IM

intramuscular

INR

international normalized ratio

IT

information technology

iu

international units

IUCD

copper intrauterine device

IUD

intrauterine device

IUS

progestogen-containing intrauterine system

IV

intravenous

IVP

intravenous pyelogram

JSA

Jobseeker’s Allowance

JVP

jugular venous pressure

K+

potassium

kg

kilograms

KUB

kidney, ureters, and bladder X-ray

L

litres

LA

local anaesthetic

LBBB

left bundle branch block

LES

Local Enhanced Service(s)

LFT

liver function tests

LH

luteinizing hormone

LIF

left iliac fossa

LMP

last menstrual period

LMWH

low molecular weight heparin

LRTI

lower respiratory tract infection

LTOT

long-term oxygen therapy

LUQ

left upper quadrant

LVF

left ventricular failure

xv

xvi Abbreviations used in the text LVH

left ventricular hypertrophy

m

metres

mane

in the morning

MAOI

monoamine oxidase inhibitor

M,C&S

microscopy, culture, and sensitivity

MCP

metacarpophalangeal

MCV

mean cell volume

MDI

metered dose inhaler

mg

milligrams

MHRA

Medicines and Healthcare products Regulatory Agency

MI

myocardial infarction

min

minutes

mL

millilitres

MMR

measles, mumps, and rubella

MND

motor neurone disease

mmHg

millimetres of mercury

mo

months

MRI

magnetic resonance imaging

MS

multiple sclerosis

MSU

midstream urine

Na

sodium

NAAT

nucleic acid amplification test

NES

National Enhanced Service

NHS

National Health Service

NI

National Insurance

NICE

National Institute for Health and Care Excellence

nocte

at night

NSAID

non-steroidal anti-inflammatory drug

NSTEMI

non-ST elevation myocardial infarction

O2

oxygen

OA

osteoarthritis

OCD

obsessive–compulsive disorder

od

once daily

OOH

out-of-hours

+

Abbreviations used in the text

OTC

over-the-counter

PAN

polyarteritis nodosa

PCI

percutaneous coronary intervention

PCO

primary care organization

PD

Parkinson’s disease

PE

pulmonary embolus

PEFR

peak expiratory flow rate

PET

pre-eclamptic toxaemia

PHCT

primary healthcare team

PIP

Personal Independence Payment

PIPJ

proximal interphalangeal joint

PMH

past medical history

PMS

Personal Medical Services

PN

practice nurse

po

oral

PO4

phosphate

POP

progesterone-only pill

PPH

post-partum haemorrhage

PR

per rectum

prn

as needed

qds

four times daily

QOF

Quality and Outcomes Framework

RA

rheumatoid arthritis

RBBB

right bundle branch block

RCGP

Royal College of General Practitioners

RCOG

Royal College of Obstetricians and Gynaecologists

RhD

rhesus factor

RIF

right iliac fossa

RTA

road traffic accident

RUQ

right upper quadrant

s

seconds

sc

subcutaneous

SLE

systemic lupus erythematosus

SOL

space-occupying lesion

3–

xvii

xviii Abbreviations used in the text SNRI

serotonin and noradrenaline reuptake inhibitor

SSRI

selective serotonin reuptake inhibitor

stat

immediately

SpO2

peripheral oxygen saturation

STEMI

ST elevation myocardial infarction

STI

sexually transmitted infection

SVC

superior vena cava

TB

tuberculosis

TCA

tricyclic antidepressant

tds

three times daily

TFT

thyroid function tests

TIA

transient ischaemic attack

u

units

U&E

urea and electrolytes

UC

ulcerative colitis

URTI

upper respiratory tract infection

US(S)

ultrasound scan

UTI

urinary tract infection

VF

ventricular fibrillation

VSD

ventriculoseptal defect

VT

ventricular tachycardia

WCC

white cell count

wk

weeks

y

years

0 All other abbreviations are defined in the text on the page in which they appear. Elderly care and child health flags: conditions peculiar to children or elderly people, or in which management varies for these groups, are flagged as follows:

Child health

Elderly care

Chapter1

What is general practice? What is general practice?  2 General practice in theUK  4 Good medical practice forGPs  6 Stress in general practice  8 General practice within the widerNHS  10 Organizations important to general practice  12 Practice in other countries  14

1

2

chapter 1

What is general practice?

What is general practice? ‘Generalism describes a philosophy of practice which is person, not disease, centred; continuous, not episodic; integrates biotechnical and biographical perspectives; and views health as a resource for living and not an end in itself.’1 In the early 19th century, when apothecaries, physicians, and surgeons provided medical care, the term ‘general practitioner’ became applied to apothecaries taking the Membership Examination of the Royal College of Surgeons of England. Over the past 60y, general practice has established itself as the cornerstone of most national healthcare systems. In so doing, general practitioners (GPs or family physicians) have shown the intellectual framework within which they operate is different from, complementary to, but no less demanding than that of specialists. What is medical generalism?  The RCGP defines medical generalism as:‘An approach to the delivery of healthcare that routinely applies a broad and holistic perspective to the patient’s problems.’ It involves: • Seeing the person as a whole and in the context of his/her family and wider social environment • Using this perspective as part of the clinical method and therapeutic approach to all clinical encounters • Being able to deal with undifferentiated illness and the widest range of patients and conditions • In the context of general practice, taking continuity of responsibility for people’s care across many disease episodes and overtime • Also in general practice, coordinating care as needed across organizations within and between health and socialcare The role of the GP  In the UK, >90% of the population is registered with a GP. GPs diagnose illness, treat minor illness within the community, promote better health, prevent disease, certify disease, monitor chronic disease, and refer patients requiring specialist services. General practice is the primary point of access to healthcare services. Although patients have an average of 5.5 consultations with their GP every year in the UK, only 1 in 20 consultations results in a secondary care referral. Everything else is dealt with in the primary care setting. To do this, GPsmust: • Have a working knowledge of the whole breadth of medicine • Maintain ongoing relationships with their patients—they are the only doctors to remain with their patients through sickness and health • Focus on patients’ response to illness rather than the illness itself, taking into account personality, family patterns, and the effect of these on the presentation of symptoms • Be interested in the ecology of health and illness within communities and in the cultural determinants of health beliefs,and • Be able to draw on a far wider range of resources than are taught in medical school, including intuition, knowledge of medicine, communication skills, business skills, and humanity   Reeve J (2010) Protecting generalism—moving on from evidence-based medicine? BJGP 60:521.

1

What is general practice?

In addition to day-to-day medical care of their patients, GPs in the UK have a number of additionalroles: • Gatekeeping GPs control access to hospital-based services, enabling cost-effectivecare • Navigating GPs work with patients/carers to guide them effectively and safely through the healthcare system • Service redesign and improvement GPs manage service provision within their own practices, and beyond their practice boundaries • Research GPs need critical appraisal skills to understand and apply relevant evidence to inform clinical decision making. They need to be competent in collecting and analysing data for service improvement, and must collaborate effectively in primary care-based research • Education GPs can be effective teachers in a wide range of contexts, educating patients, practice staff, medical students and junior doctors, fellow GPs, and the general public • Leadership Many GPs have leadership roles—in their own practices, within their localities, or nationally What is the difference between GPs and specialists?  Marshall Marinker contrasted the role of generalists and specialists as shown in Table1.1 Table1.1  Differences between GPs and specialists GPs

Specialists

Exclude the presence of serious disease

Confirm the presence of serious disease

Tolerate uncertainty—managing patients with undifferentiated symptoms

Reduce uncertainty—investigating until a diagnosis is reached

Explore probability seeing patients from a population with a relatively low incidence of serious disease

Explore possibility seeing a preselected population of patients with a relatively high incidence of serious disease

Marginalize danger—recognizing and acting on danger signs even when diagnosis is not certain

Marginalize error—ensuring accurate diagnosis and treatment

To perform their roles well, GPs must show empathy for their patients; engagement and commitment to involve themselves in every aspect of patient care; appreciation of the limits of their skills and expertise; and professionalism in their dealings with both patients and colleagues.

Further information Independent Commission on Generalism Guiding patients through complexity:modern medical generalism (2011) M www.rcgp.org.uk RCGP Medical Generalism:Why expertise in whole person medicine matters (2012) M www.rcgp.org.uk Simon C (2009) From generalism to specialty:a short history of general practice. InnovAiT 2:2–9. Marinker M (2009) General practice and the new contract. In:Bevan G, Marinker M (eds) Greening the White Paper. London: Social Marketing Foundation.

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chapter 1

What is general practice?

General practice intheUK Today, along with opticians, dentists, and pharmacists, GPs form the ‘front line’ of the NHS in the UK, providing primary medical care and acting as ‘gatekeepers’ to the secondary care system. Workload ~97% of the British population are registered with a GP. Patients register with a practice of their choice in their area—whole families are often registered with the same practice. Once registered, patients stay with that practice for an average of 12y. GPs carry out ~300million consultations/y in England alone—82% at the surgery and 4% at the patient’s home. 70% of the GP’s total workload is spent with a patient, while >20% is currently spent on administration. Working hours Standard working hours are 8 a.m. to 6.30p.m. on normal working weekdays for GMS and most PMS practices although this is currently under review with a proposed change to 8 a.m.–8 p.m. cover 7 days a week. Practices may provide ‘extended hours’ as a DES (b p.21); to qualify, the practice must provide 30min of additional opening time/1,000 registered patients at times agreed with the PCO according to local needs. Some practices also provide OOH care (b p.21). How workload is distributed between individual doctors and PHCT staff is a matter for each practice to decide. Primary care provider Term used to designate any organization providing NHS primary care services. Primary care contracts  The provider contract with the local PCO defines services primary care providers will provide, standards to achieve and payments they will receive. Currently, there are 4 contracttypes: • General Medical Services (GMS) (b p.20) • Personal Medical Services (PMS) (b p.28) • Alternative Provider Medical Services (APMS) (b p.29) • Primary Care Led Medical Services (PCLMS) (b p.29) Primary care performer list List of all doctors deemed competent to provide primary medical care held by thePCO. Partnership Group of self-employed contractors working together for mutual benefit. Apartnership can become a primary care provider as long as ≥1 partner is a GP. Although traditionally partnerships are made up of GPs only, practice managers, nurses, allied health professionals, and pharmacists can be included within partnerships. Independent contractor status Around half of all GPs in the UK work as independent contractors, providing core primary healthcare services and additional services as negotiated within their contract. As such, these GPs are self-employed, running small businesses or practices. They have management responsibilities for staff, premises, and equipment. Since most GPs receive a profit share, the amount each GP is paid depends not only on income to the practice, but also expenditure: Income • Private work Includes:private appointments (e.g. clinical assistant, industrial appointments); insurance examinations/reports; private medical examinations and certificates (e.g. HGV licence applications) • Income from the NHS GMS, PMS, or APMS contractwork

General practice intheUK

Expenditure • Running costs of the practice Staff salaries; premises (rent, rates, repairs, maintenance, insurance); service costs (heating, water, electricity, gas and telephone bills, stationery and postage); training costs,etc. • Capital expenses Purchase of new medical and office equipment

Salaried GP AGP employed by a PCO, practice, or alternative provider

of medical services (APMS). PCOs and GMS practices are bound by a nationally agreed model contract, with a salary within a range set by the Review Body. PMS practices can make their own arrangements. Salaried posts have advantages for those who do not want to commit to long-term working within one practice or become involved with managerial tasks. Pay tends to be less than that of independent contractors. GP retainer Provides an opportunity for doctors with other commitments to maintain medical skills before returning to full- or part-time employment at a later date (usually within 5y). Practices approved for the retainer scheme must provide adequate education, supervision, and support. Members of the schememust: • Have ‘licence to practise’ (b p. 69) and maintain their GMC registration • Work ≥12, but ≤208 paid service sessions a year (one session = 3.5h); most work 2–4 sessions/wk • Do ≥28h of educational sessions/y and take a professional journal

Freelance GP or locum Works for practices or PCOs by a regular or intermittent arrangement or by providing medical cover on a one-off basis. Tends to be self-employed and charge on a sessional basis. Long-term loc*ms should make their own pension provision or apply to join the NHS scheme. Some freelance GPs work from ‘locum chambers’ with administrative support to help with bookings and payments. GP with special interest (GPwSI) bp.70 GP registrar GP in 3rd/4th year of specialty training  bp.64 Practice boundaries Practices set geographical boundaries around

their practices agreed with their PCOs. Currently, practices only accept new patients onto the practice list who live within that boundary. In England, practice boundaries for patient registrations will be abolished after October 2014. However GPs will not be required to do home visits for patients living outside their practice boundaries; these will become the responsibility of NHS England Local Area Teams. Practice lists  All patients registered with a particular primary care provider. Lists may be open (accepting new patients) or, by agreement with the PCO for a set period of time, closed to new patients bp.46. Polyclinics  Also referred to as ‘Darzi centres’ are found in urban centres throughout the UK. They may be owned and run by the NHS, large GP practices, private companies, or Foundation Trusts. Key features: • Large premises serving up to 50,000 patients and housing up to 25GPs • GP services alongside other health services, e.g. dentists, pharmacists • Extended services—consultant outpatient appointments, physiotherapy, routine diagnostic services, e.g. ECG,X-ray • Extended opening—urgent care 18–24h/d and routine GP appointments in the evenings and at weekends

5

6

chapter 1

What is general practice?

Good medical practice forGPs GMC duties of a doctor1 • Make the care of your patient your first concern • Protect and promote the health of patients and the public • Provide a good standard of practice andcare • Keep your professional knowledge and skills up todate • Recognize and work within the limits of your competence • Work with colleagues in the ways that best serve patients’ interests • Treat patients as individuals and respect their dignity • Treat patients politely and considerately • Respect patients’ right to confidentiality • Work in partnership with patients • Listen to patients and respond to their concerns and preferences • Give patients the information they want or need in a way they can understand • Respect patients’ right to reach decisions with you about their treatment andcare • Support patients in caring for themselves to improve and maintain their health • Be honest and open and act with integrity • Act without delay if you have good reason to believe that you or a colleague may be putting patients atrisk • Never discriminate unfairly against patients or colleagues • Never abuse your patients’ trust in you or the public’s trust in the profession You are personally accountable for your professional practice and must always be prepared to justify your decisions and actions.

Good medical practice forGPs2 • Good clinical care Provide best possible clinical care for patients • Maintaining good medical practice Monitor, review, and continuously strive to improve performance of yourself and your practice • Teaching and training, appraising and assessing bp.60 • Relationships with patients Communicate with and listen to views and opinions of your patients; use terms/information they can understand; respect their privacy and dignity at alltimes • Working with colleagues Ensure effective communication channels within/outside the practice; ensure an environment for personal/ professional development for everyone working within the practice • Probity Behave in a proper fashion, ensuring honesty and openness in all matters. Avoid conflicts between personal and professional roles. Research bp.82 • Health GPs must be able to perform their roles to an adequate standard and be safe to practise. Concerns about performance bp.69   Reproduced with permission of theGMC   Summarized from Good medical practice for GPs (2008) M www.rcgp.org.uk

1 2

Good medical practice forGPs

Continuity of care  Apatient seeing the same healthcare worker over time. In the UK, this has been the norm, but continuity of care is becoming less available. Reasons for continuity of care Apractitioner’s sense of responsibility toward his/her patients i with duration of relationship and number of contacts. Continuity builds trust, creates a context for healing, and i practitioner’s and patient’s knowledge of each other. Evidence: • i patient and doctor satisfaction • i compliance • i uptake of preventive care,and • Better use of resources (time spent in the consultation, discriminatory use of laboratory tests, and admission to hospitals) Patients’ desire for personal care depends on the reason for the encounter. Most find it important to see their own GP for serious medical conditions and emotional problems. Reasons why continuity of care is becoming less available Problems balancing accessibility, flexibility, and continuity ofcare: • Doctor factors Flexible careers, special interests, and managerial responsibilities all limit the availability of GPs to their patients • Patient factors 24h society, in which patients want to be seen at their convenience rather than when their GP is available, makes it impossible to maintain continuous care. For minor problems and emergencies, patients do not mind who they see—as long as they see someone who can deal with their problem quickly • System factors Changing roles—nurse practitioners and other healthcare professionals commonly take on tasks which used to be done by GPs; clinical governance structures mean that patients with particular conditions are managed in clinics specifically for those conditions within the practice; other primary healthcare providers, e.g. NHS 111, walk-in clinics, and separate out-of-hours cover arrangements, further fragmentcare Rationing  Afull discussion on rationing healthcare is beyond the scope of this handbook, but, with continued innovation, rising demand, and limited resources, it will become an increasingly important factor in medicine worldwide. To some extent, there is already rationing by default—medicines and certain treatments are not provided on the NHS or have very long waiting lists. Government bodies, such as NICE, evaluate services and develop guidelines for healthcare professionals about medicines and services which are both clinically and cost-effective. Inevitably, this will mean that some groups will feel they are being deprived of the treatment they require. It will remain a contentiousissue.

Further information Appraisal and revalidation bp.68 GPC/RCGP  Good medical practice for GPs (2008) M www.rcgp.org.uk GMC  Duties of a doctor M www.gmc-uk.org

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Stress in general practice Increasing stress is a feature of society as a whole. GPs score twice the national average on stress test scores. Similar figures are seen if anxiety scores are used, and 1 in 4 GPs are classed as suffering from depression if depression screening tools are used. Burnout describes the syndrome of emotional exhaustion, depersonalization, low productivity, and feelings of low achievement. Studies of British GPs consistently find significant numbers of GPs in all age groups are affected. Causes of stress in general practice Insecurity about work (particularly changes in NHS structure and complaints), isolation, poor relationships with other doctors, disillusionment with the role of GPs, changing demands, work–home interface, demands of the job (particularly time pressure, problem patients, and emergencies during surgery hours), patients’ expectations, and practice administration.

Roots of stress  Many of the main stressors for GPs appear to be created or perpetuated by doctors’ own policies:overbooking patients, starting surgeries late, accepting commitments too soon after surgeries are due to finish, making insufficient allowances for extra emergency patients, and allowing inappropriate telephone or other interruptions. Higher than average pressure scores occur in doctors with fast consultation rates compared to those with slowerrates. General characteristics of a stressed person at work are  Lack of

concentration, poor timekeeping, poor productivity, difficulty in comp­ rehending new procedures, lack of cooperation, irritability, aggression, withdrawal behaviour, resentment, i tendency to make mistakes, and resistance to change.

Effects ofstress

• Effects on clinical work One study showed frustrated doctors are more willing to take undesirable short cuts in treating patients; another that those doctors with negative feelings of tension, lack of time, and frustration have poor clinical performance (measured by an i prescription rate and lack of explanation to patients) • Effects on practices Stress has effects on the practice too, resulting in mistakes, arguments or angry outbursts, poor relationships with patients and staff, increased staff sickness and turnover, and accidents • Effects at home Stressed GPs may develop problems in their relationships with their partners and family at home, becoming uncommunicative at home or work, and more withdrawn and isolated Experience of stress does not necessarily result in damage. The extent of stress necessary to d performance or satisfaction levels will depend on the doctor’s personality, biographical factors, and coping methods, but a concurrent illness or coexisting life event may have additive effects and can i vulnerability to stress or d ability tocope. Alcohol  Doctors commonly use alcohol as a coping method for stress. The BMA estimates 7% of doctors are addicted to alcohol and/or other chemical substances, with half of those addicted to alcoholalone.

Stress in general practice

Interventions and solutions

• Improve your working conditions, e.g. longer booking intervals for patient consultations; develop a specialist clinical or academic interest within or outside the practice; learn to decline extra commitments. GPs with high stress levels do not necessarily have low morale, but there is a close correlation between levels of job satisfaction and morale—job satisfaction seems to protect against stress • Look at your own behaviour and attitudes Stop being a perfectionist; resist the desire to control everything; don’t judge your mistakes too harshly • Look after your own health and fitness Set aside time for rest and relaxation; make time for regular meals and exercise • Allow time for yourself and your family Do not allow work to invade family time. Consider changes in working arrangements to allow more time for leisure and family • Don’t be too proud to ask for help As well as formal channels for seeking help, there are several informal doctor self-help organizations and counselling services (see Useful contacts)

Chronic stress bp.1002 Useful contacts Occupational Health Services  Available to GPs whose health is causing a performance concern. BMA  Doctors for Doctors Service and BMA Counselling Service. Provides members and their families (normally resident with them) with help, counselling, and personal support. Also produces a ‘doctors’ health and well-being’ webpage F 0845 9 200 169 M www.bma.org.uk British Doctors and Dentists Group  Support group of recovering medical and dental drug and alcohol users. Students are also welcomed. Gives confidential help and advice through a local recovering doctor or dentist. F 0779 2819966 Cameron Fund  Provides help and support solely to GPs and their dependants. It can meet needs that vary from those of the elderly in nursing homes to young, chronically sick doctors and their families, and those suffering from relationship breakdown or financial difficulties following the actions of professional regulatory bodies. F 020 73880796 Sick Doctors Trust  Aconfidential intervention and advisory service for alcohol- and drug-addicted doctors, run by doctors for doctors. 24h Helpline: F 0370 444 5163 M www.sick-doctors-trust.co.uk Doctors’ Support Network (DSN)  Aims to raise awareness of mental health concerns, encourage, doctors to look after their mental health and to seek help early. Confidential, anonymous support service, allowing doctors to talk about issues affecting them, whether mental health, work problems, or anything else F 0844 3953010 Royal Medical Benevolent Fund  Provides specialist information and advice, and necessary financial assistance due to age, ill health, disability, or bereavement. F 020 85409194

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General practice within the widerNHS In 1948, the National Health Service (NHS) was formed, giving free healthcare for the entire population of the UK paid for by the taxpayer. The NHS is now the largest organization in Europe. The structure of the NHS varies from country to country within theUK. England  (See Figure1.1) • Secretary of State for Health Head of the NHS responsible to Parliament • Department of Health (DH) Sets overall health policy in England, is headquarters for the NHS, and is responsible for developing and putting policy into practice • Monitor Independent regulator of healthcare in England • NHS Commissioning Board Key link between the DH and NHS. Advises Clinical Commissioning Groups (CCGs) and holds them to account. Manages the budgetallocated to the NHS, distributing it to CCGs, and also directly commissions primary care and national services • Healthwatch England Part of the Care Quality Commission. Provides feedback from service users through a network of Local Healthwatch organizations to the Secretary of State for Health, NHS Commissioning Board, Monitor, and local authorities • Clinical Commissioning Groups (CCGs) Cornerstone of the NHS— responsible at a local level for planning, providing, and commissioning health services from service providers, and improving the health/ well-being of the local population • Health and Wellbeing Boards Work with patients, CCGs, and local authorities to develop a Joint Strategic Needs Assessment (JSNA) in order to improve health/well-being of the local population. The JSNA then influences commissioning decisions across health, public, health and social care, thus promoting integrated health/socialcare • NHS Trusts Provide hospital and specialist community services Government Department of Health; Secretary of State for Health NHS Commissioning Board Local Clinical Commissioning Groups Secondary care NHS Trusts • Acute and specialized hospital services • Mental health services

Figure1.1  Structure of the NHS in England

Primary care GP services

PATIENT CARE

General practice within the widerNHS

Northern Ireland  The Department of Health, Social Services and Public Safety (DHSSPS) is responsiblefor: • Health and Social Care Board Agent of the DHSSPS in planning, commissioning and purchasing services for the residents of Northern Ireland, including primary care services • 6 Health and Social Care Trusts Cover the whole of Northern Ireland (Belfast, Northern, Southern, Western, South Eastern, and Northern Ireland Ambulance Service) and directly provide services to people in theirareas • 19 Health Agencies Provide national services, e.g. Public Health Agency; Blood Transfusion; Northern Ireland Cancer Screening • Patient and Client Council Provides patient/client feedback to shape services Further information M http: www.n-i.nhs.uk

Scotland • Scottish Government Health Directorate Responsible both for NHS Scotland and for the development and implementation of health and community care policy • NHS Boards Health services are delivered through 14 regional NHS Boards. These Boards provide strategic leadership and performance management for the entire local NHS system in their areas and ensure that services are delivered effectively and efficiently. NHS Boards are responsible for the provision and management of the whole range of health services in an area, including hospitals and general practice • Special Boards and Public Health Body Scotland has seven Special Boards delivering services across the whole of Scotland e.g. Scottish Ambulance Service; NHS24. In addition, there is one Public HealthBody Further information M www.show.scot.nhs.uk

Wales  The NHS is Wales’ largest employer (7% workforce). • National Advisory Board Responsible for providing independent advice to the Welsh Minister for Health and Social Services • National Delivery Group The Chief Executive, NHS Wales, is responsible for providing the Welsh Minister for Health and Social Services with policy advice and for exercising strategic leadership and management of the NHS. To support this role, the Chief Executive chairs a National Delivery Group, forming part of the Department for Health and Social Services (DHSS). This group is responsible for overseeing the development and delivery of NHS services acrossWales • Local Health Boards (LHBs) 7 boards secure/deliver healthcare services in their areas. LHBs are responsible for commissioning GP services from practices, and community and secondary care services • NHS Trusts 3 Trusts deliver services that operate throughout Wales. These are:Public Health Wales, Welsh Ambulance Services NHS Trust, Velindre NHS Trust offering cancer care services, and the Welsh Blood Service • Community Health Councils 7 statutory lay bodies (one for each LHB) that represent the interests of the public Further information M http: www.wales.nhs.uk

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Organizations important to general practice British Medical Association (BMA)  Voluntary professional association and independent trade union of doctors. >80% of UK doctors are members. Also runs a publishing house, producing books and journals (including the BMJ); negotiates doctors’ pay and terms of service; provides advice about matters related to work practice; provides educational and research facilities, accommodation, dining facilities, and financial services. The General Practitioners Committee (GPC) is a subgroup. Further information F 020 7387 4499 M www.bma.org.uk

Care Quality Commission (CQC) Aindependent public body. Its functions areto: • Assess management, provision and quality of health and social care in England (including GP practices) • Regulate the independent healthcare sector through registration, annual inspection, monitoring complaints, and enforcement • Publish information about the state of health and socialcare • Consider complaints about NHS organizations that the organizations themselves have not resolved • Coordinate reviews and assessments of health and social care and carry out investigations of serious failures in the provision ofcare Further information M www.cqc.org.uk

General Medical Council (GMC) Licenses doctors to practise medicine in the UK. It investigates complaints against doctors and has the authority to revoke a doctor’s licence, if appropriate. It also monitors standards of undergraduate, postgraduate, and continuing medical education and provides information about good medical practice. Further information M www.gmc.org.uk

General Practitioners Committee (GPC) BMA committee with authority to deal with all matters affecting NHS GPs, representing all doctors in general practice, whether or not they are a member of the BMA. The committee is recognized as the sole negotiating body for general practice by theDH. Further information M www.bma.org.uk

Independent Complaints Advocacy Service (ICAS) Supports

patients and their carers wishing to pursue a complaint about their NHS treatment orcare.

Local Medical Committee (LMC)  Committee of GPs representative of GPs in their area. All GPs (including loc*ms and salaried doctors) are represented by LMCs. Functions: • Statutory Consultation regarding administration of the GMS and PMS contracts; involvement with disciplinary and professional conduct committees; representation of GPs as awhole • Non-statutory Advice on all matters concerning GPs; communication between GPs; links with other bodies; helping individualGPs

Organizations important to general practice

National Association of Sessional GPs (NASGP)  Acts as a voice and resource for all NHS GPs who work independently of the traditional ‘GP principal’ model. This includes GP loc*ms, retainers, salaried GPs, and GP assistants. Further information M www.nasgp.org.uk

National Institute for Health and Care Excellence (NICE)  Special

authority which aims to provide patients, health and social care professionals, and the public with authoritative guidance on ‘best practice’ and thus improve the quality/consistency of health and social care services. It evaluates health technologies and reviews management of specific conditions. Further information M www.nice.org.uk

NHS Business Services Authority (NHSBSA)  bp.141 Patient Advice and Liaison Service (PALS) Provided by all

healthcare organizations running hospitals, GP or community health services. Equivalent service in Scotland is the Patient Advice and Support Service (PASS). Aimsto: • Advise and support patients, their families, and carers • Provide information on NHS services • Listen to and record concerns, suggestions, or queries. PALS can liaise directly with NHS staff and managers regarding patients’ concerns • Help to sort out problems quickly • Direct NHS users to sources of independent advice and support, e.g. Independent Complaints Advocacy Services (ICAS)

Royal College of General Practitioners (RCGP) Founded to ‘encourage, foster and maintain high standards within general practice and to act as the voice of GPs on issues concerned with education, training, research and standards’. Services include: • Publishing—books and journals including British Journal of General Practice and InnovAiT • Education—online learning, courses, conferences • Revalidation support, including e-portfolio • Representation of GPs at national and international levels • International collaboration, including international membership section • Support for specific groups, e.g. First5 (for GPs within 5y of their Certificate of Completion of Training); AiT Committee for GPs in training Statutory responsibilities include: • Developing and updating the GP training curriculum • Setting and managing the UK licensing examination for general practice • Managing certification and recertification 3 grades of membership: • Members are entitled to speak and vote at meetings, and to use the designation MRCGP (b p.66) • Fellows Highest grade of membership; holders use the designation FRCGP (b p.66) • Associates For doctors still in training. Associates can participate in College activities but cannot vote or use the designationMRCGP Further information M www.rcgp.org.uk

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Practice in other countries It is beyond the scope of this book to discuss different systems of healthcare and practice regulations outside the UK; however, in most countries, there is a registration body (usually termed the ‘Medical Council’) which ensures doctors are qualified and fit to practise; an organization representing the interests of the medical profession generally (often termed the ‘Medical Association’); and separate specialist bodies representing the interests of family practitioners. Details can be obtained from the following websites:

International Directory of Medical Regulatory Authorities  Lists worldwide medical regulatory bodies and contact details. M www.iamra.com

World Organization of Family Doctors (WONCA)  Includes a

list of member organizations and contact details. M www.globalfamilydoctor.com

European Union of General Practitioners/Family Physicians  Gives overview of different healthcare systems in member

states and contacts for member organizations. M www.uemo.eu

Medical Association of South East Asian Nations (MASEAN)  Contains contact details for Medical Associations in Brunei, Cambodia, Indonesia, Laos, Malaysia, Myanmar (Burma), Philippines, Singapore, Thailand, and Vietnam. M www.masean.org

Country-specific information Australia • Australian Medical Council M www.amc.org.au • Australian Medical Association M www.ama.com.au • Royal Australian College of General Practitioners M www.racgp.org.au Canada • Medical Council of Canada M www.mcc.ca • Canadian Medical Association M www.cma.ca • College of Family Physicians of Canada M www.cfpc.ca China • Chinese Medical Association M www.cma.org.cn HongKong • Medical Council of Hong Kong M www.mchk.org.hk • Hong Kong Medical Association M www.hkma.org • Hong Kong College of Family Physicians M www.hkcfp.org.hk India • Medical Council of India M mciindia.org • Indian Medical Association M www.ima-india.org Ireland(Eire) • Irish Medical Council M www.medicalcouncil.ie • Irish Medical Organization M www.health.ie • The Irish College of General Practitioners M www.icgp.ie

Practice in other countries

Japan • Japan Medical Association M www.med.or.jp • Japan Primary Care Association M www.primary-care.or.jp New Zealand • Medical Council of New Zealand M www.mcnz.org.nz • New Zealand Medical Association M www.nzma.org.nz • Royal New Zealand College of General Practitioners M www.rnzcgp.org.nz Pakistan • Pakistan Medical and Dental Council M www.pmdc.org.pk Singapore • Singapore Medical Council M www.smc.gov.sg • Singapore Medical Association M www.sma.org.sg • College of Family Physicians Singapore M www.cfps.org.sg SouthAfrica • Health Professions Council of South Africa M www.hpcsa.co.za • South African Medical Association M www.samedical.org • South African College of Family Physicians M www.collegemedsa.ac.za USA • Educational Commission for Foreign Medical Graduates (ECFMG) Mwww.ecfmg.org • American Medical Association (AMA) M www.ama-assn.org • Federation of State Medical Boards M www.fsmb.org • American Board of Family Practice M www.abfp.org • American Academy of Family Physicians M www.aafp.org

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Chapter2

Contracts Partnership agreements  18 General Medical Services Contract  20 GP pay under the GMS Contract  22 Carr-Hill Allocation Formula  24 Quality and Outcomes Framework  26 Other provider contracts  28 Commissioning  30

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Contracts

Partnership agreements Partnership disputes are common. Aproperly drafted partnership agreement may prevent disputes and, if they do occur, may lessen their impact.

Partnership atwill A partnership without an up-to-date written agreement is a ‘partnership at will’, governed by the 1890 Partnership Act. A‘partnership at will’ is a very unstable situationas: • All partners are deemed to have equal profit shares, unless there is clear evidence to the contrary • Decisions are made by simple majority • Notice may be served by any partner on the others without their prior knowledge or consent • Dissolution of the partnership may take immediate effect, and no reason needs be given to justifyit • Dissolution may result in the forced sale of all partnership assets (including the surgery premises) and redundancy of allstaff • There is nothing to prevent any partner, or group of partners, from immediately forming a new practice/partnership to the exclusion of the other partner(s) once the practice is dissolved Partnership agreements Should be drawn up every time a new partner joins or leaves a practice. Employed doctors and retainers also require contracts of employment. An agreement checklist is included in Box 2.1. Detailed guidance is produced by the BMA, and further guidance can be obtained from local BMA offices andLMCs. Partnership disputes However good a partnership agreement is, disputes still occur. Advice on partnership and employment matters is available from the BMA and LMC, and the BMA also provides conciliation services (contact local office). Legal battles are expensive, and the BMA will not fund partnership disputes. Try to resolve matters amicably. Discrimination It is unlawful for any partnership to discriminate on grounds of age, gender, marital status, colour, race, nationality (including citizenship), ethnic or national origins when appointing a new partner or in the way they treat an existing partner. The BMA will consider backing GPs to take such matters to industrial tribunals—contact the local office. Applications should be made on forms available via local Job Centres and must be made within 3mo of the last act of discrimination. Contracts of employment for salariedGPs Model terms and conditions of service for a salaried GP and a model offer letter of employment are available from the BMA or DH websites. Nationally agreed salary scales apply and are compulsory for GMS, but not PMS, practices. Responsibilities towards employedGPs bp.36

Partnership agreements

Box 2.1  Partnership agreement checklist • Business detail Purpose of the business; premises and basis of occupation. If premises are owned by the partners, state procedure for valuation, payment of the retiring partner, and investment of the incoming partner • Assets Specify assets, their ownership, arrangements for valuation and interest payments. It is illegal to sell goodwill in NHS practices • Income and allowances Definition of practice income; allowable expenses • Profit sharing Distribution of practice NHS income and other NHS allowances; distribution of income from non-NHSwork • Accounting Accounting and banking arrangements; cheque signing; access to accounts and bank statements • Taxation Arrangements for paying tax; obligations of each partner • Pension arrangements • Retirement/suspension/expulsion Reasons for suspension/expulsion; process of suspension/expulsion; mechanisms of voluntary leaving/ retirement; division of assets in the event of retirement. May include a restrictive clause preventing the outgoing doctor working in the practice area for a period of time after leaving—seek legaladvice • Leave Holiday entitlement; basis of deciding who has holiday when; study leave; sabbatical leave; sick leave; maternity, paternity, and adoption leave; compassionateleave • Obligation NHS obligations; non-NHS work within the practice; other work outside the practice; educational activities; obligations to each other; hours ofwork • Decisions and disputes Decision-making process; process to manage disputes; process to dissolve partnership. Ensure that who pays legal fees for who in the event of a dispute is included • Correct procedure Ensure each partner has signed and dated the agreement and that their signature has been witnessed. It is recommended that each partner should take independent legal advice and not rely on the ‘practice solicitor’ for soleadvice

Further information DH M www.dh.gov.uk BMA M www.bma.org.uk Equality and Human Rights Commission F 0845 604 6610 (England); 0845 604 8810 (Wales); 0845 604 5510 (Scotland) M www.equalityhumanrights.com

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General Medical Services Contract Although there may be some differences in process in each of the four countries of the UK, the principles of the General Medical Services (GMS) Contract apply toall.

Primary Care Organization(PCO) PCOs vary across the 4 nations of the NHS but, for the purposes of GP Contracts, arethe: • NHS Commissioning Board in England (NHS England) • NHS Boards in Scotland • Local Health Boards in Wales,and • Health and Social Care Trusts in Northern Ireland

The Contract The GMS Contract is a contract made between an individual practice and a PCO. All the partners of the practice, at least one of whom must be a GP, have to sign the contract. It includes: • National terms applicable to all practices (the ‘Practice Contract’) • Adescription of which services will be provided by that practice,i.e. - Essential services - Additional services if not optedout - Out-of-hours cover if not optedout - Enhanced services if optedin • Alevel of quality of essential and additional services that the practice ‘aspires’to • Support arrangements, e.g. for IT and premises • Asummary of the total financial resources Essential services Services that all practices must undertake. These include: • Day-to-day medical care of the practice population Health promotion, management of minor and self-limiting illness, and referral to secondary care services and other agencies as appropriate, • General management of patients who are terminally ill,and • Chronic disease management Additional services  Services that the practice will usually undertake but may ‘opt out’ of. If the practice opts out, the PCO takes responsibility for providing the service instead. The practice then receives a d global sum payment. The services includedare: • Cervical screening Opting out results in a 1.1% d in globalsum • Contraceptive services Opting out l 2.4% d in globalsum • Vaccinations and immunizations Opting out of vaccinations and immunizations altogether results in a 2% d in global sum; opting out of childhood immunizations leads to a 1% d in globalsum • Child health surveillance (excluding the neonatal check). Opting out leads to a 0.7% d in globalsum • Maternity services, excluding intrapartum care (which is an enhanced service). Opting out causes a 2.1% d in globalsum • Certain minor surgery procedures Curettage, cautery, cryocautery of warts/verrucae and other skin lesions—opting out results in a 0.6% d in globalsum

General Medical Services Contract

Out of hours (OOH)care  Practices can ‘opt out’ of providing an OOH service. The decision must be made for the whole practice— individual doctors within a practice cannot ‘opt out’ alone. The cost of opting out for a practice is 7% of the global sum. Practices that have opted out of OOH can offer surgeries or consultations within the OOH period as an extended opening hours Directed Enhanced Service by agreement with their localPCO. Enhanced services Are commissioned by the PCO and paid for in addition to the global sum payment. There are 3types: • Directed Enhanced Services (DES) Services under national direction with national specifications and benchmark pricing that all PCOs must commission to cover their population. These services change from year to year and include payment targets for childhood immunizations, influenza vaccinations, and more complex minor surgery, e.g. joint injections, incisions/excisions • National Enhanced Services (NES) These services have national minimum standards and benchmark pricing but are not directed (i.e. PCOs do not have to provide these services). Examples include:anticoagulation monitoring; treatment of drug/alcohol misuse; minor injury services • Local Enhanced Services (LES) Services developed locally to meet local needs. In England, these may be commissioned by CCGs, e.g. special services for refugees Furtherdetail Other parts of the GMS Contract covered in greater detail in this book include: • IT bp.22 • Carr-Hill Formula bp.24 • Premises bp.22 • Dispensing bp.23 • Quality payments bp.22 • GP pay bp.22 • Seniority bp.23 • Minimum Practice Income Guarantee bp.23

Further information DH The GMS Contract M www.dh.gov.uk BMA The GMS Contract and supporting documents M www.bma.org.uk NHS Employers M www.nhsemployers.org

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GP pay under the GMS Contract A total sum for GMS services is given to each primary care organization (PCO) as part of a bigger unified budgetallocation. PCOs vary across the four nations of the NHS, but, for the purposes of GP payments, they are the NHS Commissioning Board in England, NHS Boards in Scotland, Local Health Boards in Wales, and Health and Social Care Trusts in Northern Ireland.

Payment to practices comprises the following components • • • • • •

The globalsum+ Quality payments+ Enhanced services payments+ Payment for premises+ IT payments+ Dispensing payments (if applicable)

The GlobalSum  Major part of the money paid to practices. It is paid monthly and intended to cover practice running costs. It includes provision for delivery of essential services and additional/OOH services (if not opted out); staff costs; career development; and locum reimbursem*nt (e.g. for appraisal, career development, and protectedtime). Quality payments  Payment for quality is made in 2ways: • Aspiration payments Advance payments to allow practices to develop services to achieve higher quality standards. Practices agree their aspirations for quality points the following year with their PCO. Aspiration payments are made monthly alongside global sum payments and amount to roughly 70% of the points achieved in the previous year (for 2012/13, this was equivalent to 2011/12 points achieved × £133.76/point × 70% × adjustment for list size and composition) • Achievement payments Payments made for the practice’s actual achieved number of points in the Quality and Outcomes Framework (b p.26) as measured at the start of the following year. Aspiration payments that have already been received are deducted from the total (i.e. actual payment = achievement payment– aspiration pay already received) Payment for extra ‘enhanced’ services Paid to practices that

provide Directed Enhanced Services, National Enhanced Services, and/or Local Enhanced Services.

Payment for premises and information technology GP premises are funded in many different ways. The GP contract has provision to reimburse practices that rent their premises the cost of the rent, or pay practices that own their premises for the use of those premises. The PCO also reimburses all the IT costs of the practice.

GP pay under the GMS Contract

DispensingGPs  Any GP, in an area classified as rural, may apply to

dispense to any of his/her patients living >1 mile from the local pharmacy, as long as this would not render the pharmacy’s business unviable. Aseries of fees are paid for providing this service in a similar way to that in which community pharmacists are funded and in addition to the GMS globalsum.

Carr-Hill Allocation Formula  GMS resource allocation formula for

allocating funds for the Global Sum and quality payments. The formula takes the practice population and then makes a series of adjustments based on the profile of the local community, taking account of determinants of relative practice workload and costs. The resulting ‘allocation factor’ is then applied to the global sum and quality payments made to the practice—bp.24.

Minimum Practice Income Guarantee(MPIG) Designed to protect those practices that lost out when the GP Contract changed in 2004. It is the difference between the Global Sum allocation (GSA) under the new GMS Contract and the Global Sum equivalent (GSE)—the amount the practice would have earned providing the same service under the old Contract. If GSA < GSE, a correction factor (CF) is applied so that GSA + CF=GSE. MPIG is now being slowly phased out. Seniority payments  Payment system based on years of NHS service.

Superannuable income is used as a measure of that service. From 2014 this scheme has not been open to new GPs and it will end for all GPs in 2020.

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Carr-Hill Allocation Formula Geographical and social factors result in differing workload for GPs (see Table2.2). The Carr-Hill Formula allocates payment to practices on the basis of the practice population, weighted for factors that influence relative needs and costs in order to reflect the differences in workload these factors generate.

Age-sex adjustments  Older people and children 3mo and expected to last >6momore* • ≥65y and not permanently in hospital/local authority accommodation • Needs attention/supervision—higher rate if 24h care required or terminal illness*

Attendance Allowance (AA)∇

chapter 9

Table9.5 (Cont.)

Table9.6  Mobility for elderly and disabled people. Eligibility

How to Apply

Benefits gained

Blue Badge Scheme

Age >2y and ≥1 of the following: • War Pensioner’s Mobility Supplement • Higher rate of the mobility component of DLA/PIP • Motor vehicle supplied by a government health department • Registeredblind • Severe disability in both upper limbs, preventing turning of a steeringwheel • Permanent and substantial difficulty walking

Apply through local social services department 0 In most circ*mstances, the disabled person does not have to be the driver. The badge should not be used if the disabled person is not in the car M www.dft.gov.uk

Entitles holder topark: • In specified disabled spaces; • Free of charge or time limit at parking meters or other places where waiting is limited • On single yellow lines for up to 3h (no time limit in Scotland)

Motability Scheme

• Higher rate mobility component of DLA/PIPor • War Pension Mobility Supplement

Contact Motability. Application guide available at M www.motability.co.uk

Registered Charity. Mobility payments can be used to lease or hire-purchase a car, powered scooter, or wheelchair. Grants may also be available for advance payments, adaptations, or driving lessons

0 Driver may be someone else

• Higher rate mobility component of DLA/PIPor • War Pension Mobility Supplementor • Person nominated as someone who regularly drives for a disabled personor • Certain types of powered invalid carriages

Usually received automatically. If not and claiming DLA/PIP,  F 0845 7123456. If claiming War Pension, F 0800 1692277

Exemption from Road Tax

Seatbelt exemption

Certain medical conditions, e.g. colostomy—but weigh up risks of travelling without a seatbelt

Medical practitioner must complete exemption certificate

Exemption from wearing seatbelt

0 Local public transport schemes alsoexist

Pensions and benefits

Road Tax Exemption

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Benefits received

Anyone requiring a wheelchair(s) for >3mo. Short-term loan of equipment is often available via the Red Cross

Referral by GP or specialist to the wheelchair service centre. Directory of service centres is available at: M www.wheelchairmanagers.nhs.uk

Provision of suitable wheelchair Vouchers enable disabled patients to purchase their chairs privately

Occupational therapy (OT) assessment

All elderly or disabled people

Request a needs assessment by an occupational therapist via the local social services department

Enables provision of equipment and adaptations necessary to maintain an independent lifestyle

Disabled Living Centres / Disability Living Foundation

All elderly or disabled people

49 Disabled Living Centres in the UK—list available from M http://assist-uk.org

Disabled Living Centres: Look at and try out equipment, with OTs on hand to advise

Disabled Living Foundation

Disabled Living Foundation: Information on aids and adaptations

 F 0845 130 9177 M www.dlf.org.uk Telephone

People who have physical difficulty using the telephone or communication problems

British Telecom has a helpful website Mwww.bt.com/includingyou/index.html

Gadgets and services that make it easier for disabled or elderly people to use the telephone

Alarm systems

Any disabled or elderly person who is alone at times, at risk, and mentally capable of using an alarm system

Arrange via local social services or Housing Department. Alternatively, charities for the elderly have schemes (e.g. Age UK Personal Alarm F 0800 011 3846)

Enables a call for help when the phone cannot be reached

0 All purchases related to disability are VAT exempt.

Chronic disease and elderly care

Eligibility Wheelchairs

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Table9.7  Adaptations and equipment for elderly and disabledpeople

Pensions and benefits

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Cardiology and vascular disease Symptoms and signs of CVD  232 Examining the heart  234 Examination of the arterial system  236 Cardiac investigations  238 Brief guide to common ECG changes  240 Prevention of coronary heart disease  242 Estimating cardiovascular risk  244 Blood pressure measurement  246 Hypertension  248 Hyperlipidaemia  252 Angina  256 Drug treatment of angina  258 After myocardial infarction  260 Chronic heart failure  262 Management of chronic heart failure  264 Pulmonary hypertension and cor pulmonale  266 Tachycardia  268 Atrial fibrillation  270 Bradycardia  272 Infective endocarditis  274 Rheumatic fever, myocarditis, and pericarditis  276 Cardiomyopathy and heart transplant  278 Valve disease  280 Other structural abnormalities of the heart  282 Aneurysms  284 Chronic peripheral ischaemia  286 Varicose veins   288 Deep vein thrombosis  290

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Symptoms and signsofCVD Chestpain b p.1080 Blood pressure bp.246

Breathlessness or dyspnoea bp.294 Crackles in thechest bp.299

Peripheraloedema Swelling of the ankles/legs (or sacrum if bed-bound) occurs when the rate of capillary filtration > rate of drainage. • Increased capillary filtration occurs due to i venous pressure, hypoalbuminaemia, or local inflammation • Decreased drainage occurs due to lymphatic obstruction Consider whether swelling is acute or chronic, symmetrical or asymmetrical, localized, or generalized. Ask about associated symptoms, e.g. breathlessness. Treat according to cause. Causes: Acute • Fracture • Cellulitis • DVT • Acute arterial • Haematoma • Superficial thrombophlebitis ischaemia • Baker’scyst • Joint effusion/ • Dermatitis • Arthritis haemarthrosis Chronic • Gravitational oedema, e.g due to immobility—common in the elderly—advise elevation of feet above waist level when sitting, support stockings (ideally apply stockings before getting out of bed), avoid standing still. Diuretics are not a long-term solution • Chronic venous insufficiency/ • Heart failure • Hypoproteinaemia, e.g. nephrotic venous obstruction • Lipodermatosclerosis syndrome • Lymphoedema—infection, • Idiopathicoedema tumour,trauma • Reflex sympathetic dystrophy • Congenital vascular abnormalities • Post-thrombotic syndrome

Pulmonaryoedema  Accumulation of fluid in the pulmonary tissues and air spaces. Causes include:

Cardiac / vascular • Left heart failure • Mitral stenosis • MI • Hypertension • Pulmonary venous obstruction • IV fluid overload

Other • High altitude • Kidney failure • Nephrotic syndrome • Cirrhosis • Lymphatic obstruction, e.g. due totumour

Lung • PE • Pneumonia • Pneumonitis due to inhalation of toxic substances, e.g. gases, radiation

Cyanosis  Dusky blueskin. Central cyanosis  Cyanosis of mucus membranes, e.g. mouth. Causes: • Lung disease resulting in inadequate oxygen transfer (e.g. COPD, PE, pleural effusion, severe chest infection)

Symptoms and signsofCVD

• Shunting from pulmonary to systemic circulation (e.g. Fallot’s tetralogy, PDA, transposition of the great arteries) • Inadequate oxygen uptake (e.g. met- or sulf-haemoglobinaemia) Peripheral cyanosis,  e.g. cyanosis of fingers. Causes: as for central cyanosisplus • Physiological (cold, hypovolaemia) • Local arterial disease (e.g. Raynaud’s syndrome) 0 Feet can be a dusky blue colour due to venous disease. If this occurs without central cyanosis it does not imply abnormal oxygen saturation. Mitralfacies  Dusky bluish red flushing of the cheeks (a form of peripheral cyanosis) associated with a low cardiac output.

Clubbing  Loss of the angle between nail fold and plate, bulbous finger tip, and the nail fold feels boggy—b p.609. • Refer any patient with unexplained nail clubbing for urgentCXRN.

Jugular venous pressure  Observe internal jugular vein at 45° with

head turned slightly to the left. Vertical height is measured in relation to the sternal angle. Raised if >4cm. Causes ofiJVP: • Fluid overload • Pulmonary hypertension • Right heart failure andCCF • Arrhythmia—AF or atrial flutter, • SVC obstruction (non-pulsatile) complete heartblock • Tricuspid or pulmonary valve • i intrathoracic pressure e.g. disease pneumothorax, PE, emphysema Kussmaul’ssign  The JVP usually drops on inspiration along with intrathor­ acic pressure. The reverse pattern is called Kussmaul’s sign. Caused by raised intrathoracic pressure or constrictive pericarditis.

Signs of infective endocarditis • Infective Fever, weight d, clubbing, splenomegaly, anaemia • Cardiac Murmurs (particularly new murmurs) ± heart failure • Embolic Neurologic deficit due tostroke • Vasculitic Microscopic haematuria, splinter haemorrhages, conjunctival haemorrhages, Roth’s spots (retinal vasculitis), Osler’s nodes (painful lesions on finger pulps), Janeway lesions (palmar macules)

Signs of hypercholesterolaemia Cornealarcus  Whitish opaque line surrounding the margin of the cornea, separated from it by an area of clear cornea. Rarely congenital—more commonly occurs bilaterally in patients >50y (arcus senilis). Sometimes associated with i blood lipids—particularly familial hypercholesterol­ aemias. Check lipids. If lipids are normal, no treatment is needed. Xanthomata  Localized collections of lipid-laden cells. Appear as yellowish coloured lumps. Often caused by i lipids. Commonly seen on the eyelids (xanthelasma), on the skin, or in tendons (appear as mobile nodules in the tendon).

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Examining theheart Apexbeat  Normal position is in the 5th intercostal space, in the mid­

clavicular line. Moved sideways/inferiorly if the heart is enlarged (e.g. CCF) or displaced (e.g. pneumothorax). May not be palpable if the patient is obese, has hyperexpanded lungs (e.g. COPD), or a pericardial effusion. In infants/children apex beat is superior/more lateral. Parasternalheave  Detect by placing the heel of the hand over the left parasternal region. If present, the heel of the hand is lifted off the chest wall with each heart beat. Causes: usually right ventricular enlargement— rarely, left atrial enlargement. Heartsounds See Table10.2. Low/medium frequency sounds (e.g. 3rd/4th heart sounds) are more easily heard with the bell applied lightly to the skin. High-frequency sounds (e.g. 1st/2nd heart sounds and opening snaps) are more easily heard with a diaphragm. Heart murmurs  Due to abnormalities of flow within the heart and great vessels. Very common. Often incidental findings. Describedby: • Location Where heard loudest • Quality, e.g. blowing,harsh • Intensity Graded out of 6 (1—virtually undetectable; 6—heard by an observer with no stethoscope). Grades 4–6 are usually palpable (thrills) • Timing Systolic or diastolic,and • Radiation Does the murmur spread elsewhere, e.g. to axilla, carotids

• Red flag symptoms • Cyanosis • Breathlessness

• Lethargy/tiredness • Collapse

• Weight loss (or failure to thrive)

Always refer for echo. Differential diagnosis—SeeTable10.1. Table10.1  Differential diagnosis of heart murmurs Type of murmur Description Ejection systolic murmur Pansystolic murmur Early diastolic murmur Middiastolic murmur

Causes

• Flow murmurs, e.g children, pregnancy, with fever, during/after exercise •  Aortic stenosis or sclerosis (b p.280) •  Pulmonary stenosis (b p.281) •  HOCM (b p.278) • Mitral valve regurgitation/prolapse Uniform intensity (b p.280) between the two •  Tricuspid regurgitation (b p.281) heart sounds. Merges •  VSD (b p.282) with 2nd heart sound •  ASD (b p.282) •  Aortic regurgitation (b p.281) Occurs just after the •  Pulmonary regurgitation (b p.281) 2nd heart sound. High •  Tricuspid stenosis (mitral stenosis coexists) pitched. Easily missed Midway between 2nd •  Mitral stenosis (b p.280) heart sound of one beat •  Aortic regurgitation. (Austin Flint and 1st of the next. murmur—b p.281) Rumbling/low pitch

i to reach a peak midwaybetween the heart sounds

Examining theheart

Table10.2  Heart sounds, abnormalities, and theircauses Heart sound

Causes

1st heart sound Heard loudest at the apex Caused by closing of the mitral and tricuspid valves

Soft

Mitral regurgitation, low BP, rheumatic carditis, severe heart failure, LBBB

Loud

AF, tachycardia, atrial premature beat, mitral stenosis

Variable intensity

Varying duration of diastole, complete AV block

Split

RBBB, paced beat from the left ventricle, left ventricular ectopics, ASD, Ebstein’s anomaly, tricuspid stenosis

2nd heart sound

Soft

•A 2—calcification of the aortic valve, dilatation of the aortic root • P2—pulmonary stenosis

Loud

•A 2—i BP; thin patients • P2—pulmonary hypertension, ASD

Wide splitting

May be the result of early A2 or delayed P2 • Early A2—mitral regurgitation; VSD • Delayed P2—RBBB, pulmonary stenosis, ASD, right ventricular failure

Reversed splitting

A2 is delayed. P2 occurs before A2, so the split between the sounds d on inspiration Delayed A2—LBBB, systolic hypertension, HOCM, severe aortic stenosis, PDA, left heart failure

Single

Calcification of the aortic valve, pulmonary stenosis, Fallot’s tetralogy, Ebstein’s anomaly, pericardial effusion, large VSD, obesity, emphysema

Early systolic

Caused by opening of the aortic or pulmonary valves • Aortic—aortic stenosis, bicuspid valve • Pulmonary—pulmonary stenosis, pulmonary hypertension

Mid/late systolic

Mitral valve prolapse

Diastolic

Caused by opening of the mitral or tricuspid valves Silent in the healthy heart • Mitral—mitral stenosis, rapid mitral flow, e.g. PDA, VSD, severe mitral regurgitation • Tricuspid (rare)—rheumatic stenosis, ASD

Right ventricle

Loudest at lower left sternal edge. Never normal. Causes: right heart failure, tricuspid regurgitation, ASD, constrictive pericarditis

Left ventricle

Loudest at the apex when inclined to the left. Can be normal in children and pregnancy. Other causes: LVF, mitral regurgitation, anterior MI

Caused by closure of the aortic (A2) and pulmonary (P2) valves A2 and P2 split on inspiration so that P2 is heard after A2

Clicks and snaps

3rd heart sound Heard in diastole after the 2nd heart sound 4th heart sound Heard in late diastole

Maximal at the apex or lower left sternal edge. Never normal. Causes: ventricular hypertrophy or fibrosis and HOCM

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Examination of the arterialsystem The main conditions affecting the abdominal and peripheral arteriesare: • Aneurysms (b p.284) • Atherosclerosis, resulting in ischaemia of the legs and intermittent claudication, atrophic changes and/or restpain • Embolization resulting in acute ischaemia of thelimbs

Generalscheme • Look at the limbs—are there any signs of ischaemia? Are the extremities warm or cold? What colour arethey? • Examine the abdomen looking for a pulsatile mass which might suggest abdominal aortic aneurysm (b p.284). Auscultation may reveal abruit • Check the peripheralpulses • Tenderness on palpation of an abdominal aortic aneurysm suggests need for urgent operative repair.

Blood pressure  bp.246 Carotidpulse  Ask the patient to lie supine with head/neck at 45° to the horizontal. When assessing the carotid pulse, consider: Rate • Tachycardia >100bpm—bp.268 • Bradycardia 10mmHg— asthma, cardiac tamponade, pericarditis

Carotidbruits  May signify stenosis (>30%) often near the origin of internal carotid. Heard best behind the angle of the jaw. Usual cause is atheroma. Peripheralpulses Location  See Table10.3. Examination  Check whether each pulse is present. If presentcheck: • Rate • Rhythm • Amplitude • Compare pulses in the two legs/twoarms Check for radiofemoral delay—palpate radial and femoral pulses simultan­ eously—delay suggests coarctation of theaorta.

Examination of the arterialsystem

Table10.3  Location of the limbpulses Pulse Brachial

Location 72cm medial to the central point of the antecubital fossa over the elbow skin crease

Radial

7½–1cm on the radial (lateral) side of the flexor carpi radialis tendon at the wrist

Femoral

Below inguinal ligament; one-third of the way up from pubic tubercle

Popliteal

With knee flexed at right angles, palpate deep in the midline

Posterior tibial

1cm behind medial malleolus

Dorsalis pedis

Variable—on the dorsum of the foot just lateral to the tendons to the big toe 0 Many healthy people have only one foot pulse

Check for bruits over the femoral and/or carotid pulses—these indicate disturbed blood flow—usually 2° to narrowing due to atherosclerosis. • Character and waveform of the pulse should only be assessed using the femoral or carotidpulse.

Signs of ischaemia

Acute ischaemia  Acutely pale, cold, and pulseless limb—b p.1126. Refer immediately—keep the limb cool in the interim. Chronic ischaemic changes • Atrophic skin changes—pallor, cool to the touch, hairless,shiny • On lowering the leg turns a dusky blue-red colour; on elevation— pallor and venous guttering • Ulceration—check under the heel and between thetoes • Swelling suggests the patient is sleeping in a chair to avoid rest pain or, rarely, pain from deep infection • Absent foot pulses—if pulses are present, consider alternative diagnosis • Ankle–brachial pressure index0.12s wide. Last peak is above the iso-electric line in V1

May be normal; congenital heart disease (e.g. ASD), valvular heart disease, IHD, pulmonary hypertension, during SVT

QRS < 0.12s with Incomplete bundle branch abnormal-shaped QRS complex block Q-T interval abnormalities

As for RBBB or LBBB

Prolonged Q-T interval dK+, drugs (e.g. TCAs, phenothiazines, amiodarone), SAH or CVA, hypothermia Shortened Q-T interval iCa2+, digoxin

Abnormal P-waves

i P-wave amplitude (>2.5mm)

Right atrial overload—tricuspid stenosis, pulmonary hypertension, pulmonary stenosis

Biphasic P-wave in V1 Left atrial abnormality—mitral ± broad (>0.12s) often stenosis, aortic stenosis, conduction notched P-wave in ≥1 abnormalities limb lead

(Continued)

Brief guide to common ECG changes

Table 10.4 (Cont.) ECG abnormality Right ventricular Strain pattern—ST depression and T-wave hypertrophy inversion in leads V1–3. (RVH) Dominant R in V1 with narrow QRS

Possible causes Pulmonary stenosis, mitral stenosis pulmonary hypertension, ASD (± RBBB). Similar changes seen with inferior MI (T-wave upright); WPW syndrome

Left ventricular hypertrophy (LVH)

Strain pattern—STd and T-wave d in leads V4–6. Large voltages of QRS complex—sum of S in V1 and R in V5 or V6 alone >35mm

i BP, aortic stenosis, coarctation of the aorta, HOCM

Right axis deviation

b p.238

RVH/strain (e.g. following PE), cor pulmonale, pulmonary stenosis. Alone with normal QRS = left posterior hemiblock

Left axis deviation

b p.238

LVH /strain (e.g. iBP, aortic stenosis, HOCM), VSD, ASD. If occurs alone with normal QRS=left anterior hemiblock

Poor R-wave progression

Small or absent R waves L or R ventricular enlargement, in the R l mid-precordial LBBB, left pneumothorax, dextrocardia, COPD leads Right ventricular enlargement Reversed R wave progression—din R-wave amplitude from V1 l mid/ lateral precordial leads

Abnormal Q-waves

>25% of succeeding R-wave and/or >0.04s wide

Normal; left pneumothorax; dextrocardia; MI; myocarditis; hyperkalaemia; cardiomyopathy; amyloid; sarcoid; scleroderma; LVH; RVH; LBBB; WPW syndrome

ST elevation

ST segment raised >1mm above baseline

MI, Prinzmetal angina, pericarditis, ventricular aneurysm

ST depression

ST segment lowered >0.5mm below baseline

Angina, ventricular strain, drugs (digoxin, verapamil), hyperkalaemia, myocarditis, cardiomyopathy, fibrosis, Lyme disease

T-wave inversion Abnormal if inverted in leads I, II, or V4–6 U-waves

i amplitude >1mm

Inversion in precordial leads

MI (inverts 40kg/m2 • Is the person already taking antihypertensive/lipid modification therapy? • Has the person recently stopped smoking? • Is the person taking antipsychotic medication? • Are other conditions present that i risk, e.g. CKD, RA, SLE,HIV?

Secondary prevention Aims to stop progression of symptomatic CHD. 46% people who die from MI are already known to have CHD. There is strong evidence that targeting patients with CHD for risk factor modification is effective in d risk of recurrentCHD.

Prevention of coronary heart disease

Table10.5  Risk factors for heart disease Non-modifiable

Modifiable (proven benefit)

Modifiable (unproven benefit)

Age—i with age Sex—4 > 5 in those < 65y Ethnic origin—in the UK people who originate from the Indian subcontinent have i risk, Afro-Caribbeans have d risk Socio-economic position* Personal history of CVD Family history of CVD—< 55y 4; 180/110mmHg ± papilloedema ± retinal haemorrhage)or • Phaeochromocytoma (labile/postural hypotension, headache, palpitations, pallor, and excessive sweating)

Ambulatory BP monitoring(ABPM)  A BP-measuring device is worn on the body for 24h. It takes BP every 20min in the daytime and less frequently (usually hourly) at night. i BP readings on ABPM are more strongly correlated to end-organ damage than one-off measurements. ABPM is usedfor:

Blood pressure measurement

• The diagnosis of hypertension (b p.248) • Diagnosis or exclusion of ‘white coat’ hypertension • Monitoring of response to treatment • Assessing medication-related postural hypotension Interpretation  ABPM is considered abnormalif: • Average daytime ABPM is ≥135/85mmHg • Average night-time ABPM is ≥120/70mmHg 0 NICE bases the diagnosis of hypertension on average daytime ABPM only (minimum 14 readings). Night-timeABPM BP falls at night in normotensive individuals. Some hypertensive patients are described as ‘non-dippers’. This means that their BP does not fall at night and is associated with i risk of target end-organ damage. Conversely, some hypertensive patients have excessive dips in their BP at night (>10%); this is associated with i CVD events. Home BP monitoring(HBPM) Useful: • If ABPM facilities are not available within a reasonable time frame for diagnosis of hypertension • For patients who cannot tolerate ABPM,and • For monitoring of antihypertensive treatment Instructions  Patients should use a validated and calibrated BP machine. • Measure BP on at least 4—and preferably 7—consecutivedays • Measure BP 2x/d (morning and evening) when seated; take two readings >1minapart • Discard the readings taken on day 1 and take an average value of all the remaining measurements Interpretation  HBPM is abnormal if average is ≥135/85mmHg. Hypertension  bp.248 Cardiogenicshock  bp.1074 Postural hypotension  BP drops on moving from supine, or sitting, position to standing position. Presents with falls, postural dizziness, and/ or light-headedness. Measure BP supine or seated and then ask the patient to stand up. Re-measure BP after 1min standing. Standing usually causes a slight d in the systolic BP (50% of people aged >60y have hypertension. Management aims to detect and treat i BP before damage occurs.

Causes • Unknown (‘essential’)—95%; alcohol (10%) or obesity may be contributory factors • Renal disease—bp.438 • Endocrine disease—Cushing’s (both syndrome and 2° to steroids); Conn’s syndrome; phaeochromocytoma; acromegaly; hyperparathyroidism; DM—b p.355 • Pregnancy—bp.826 • Coarctation of the aorta—bp.282

Presentation • Usually asymptomatic and found during routine BP screening or incidentally. Occasionally headache or visual disturbance • May be symptoms of end-organ damage—LVH, TIAs, previous CVA/ MI, angina, renal impairment,PVD Measurement of blood pressure  bp.246 Diagnosis of hypertension  BP is a normally distributed continuous variable; each 2mmHg i in systolic BP is associated with a 7% i risk of mortality from IHD and a 10% i risk of mortality from stroke. There is no figure above which hypertension can be diagnosed definitively. Criteria currently inuseN: • Stage 1 hypertension Clinic BP ≥140/90mmHg and subsequent daytime average ABPM/HBPM ≥135/85mmHg • Stage 2 hypertension Clinic BP ≥160/100mmHg and subsequent daytime average ABPM/HBPM ≥150/95mmHg • Severe hypertension Clinic systolic BP ≥180mmHg or clinic diastolic BP ≥110mmHg • If the person has severe hypertension (systolic BP ≥180mmHg or diastolic BP ≥110mmHg) consider starting antihypertensive treatment immediately without waiting for the result of ABPM/HBPM. Refer for same day specialist assessment if suspected: • Accelerated hypertension (BP >180/110mmHg ± papilloedema ± retinal haemorrhage)or • Phaeochromocytoma (labile/postural hypotension, headache, palpitations, pallor, and excessive sweating)

Isolated systolic hypertension  Systolic BP ≥160mmHg—offer the same treatment as people with i systolic and diastolic blood pressure. Further assessment  Aims to identify target organ damage: • Examination Check heart size, heart sounds, and for heart failure; examine the fundi, looking for silver wiring, AV nipping, flame haemorrhages, and cotton woolspots • Blood tests Creatinine, electrolytes, eGFR, glucose/HbA1c, lipid profile; consider GGT if excess alcohol is a possibility

Hypertension

• Urine Dipstick for RBCs and protein; laboratory sample for albumin:creatinineratio • Cardiovascular risk estimation bp.244 • ECG ± echo (if left ventricular hypertrophy is suspected) 0 If iBP is not diagnosed but there is evidence of target organ damage (e.g. LVH, proteinuria) look for alternative causes. Education  Patients will not take tablets regularly, be motivated to change lifestyle, or turn up for regular checks if they do not understand why treating their i BP is important. Conversely, some patients who were fit and well prior to their diagnosis will assume a sick role unless it is explained that they are well and treatment is designed to stop illness developing. Back up verbal information with written information that patients can take home; reinforce information at follow-up and offer opportunities for discussion. Do not forget to warn patients about possible side effects of any medications. Lifestyleadvice  Offer lifestyle advice to all patients with a diagnosis of hypertension and those with FH of i BP. Reinforce advice with written information: • Offer smoking cessation advice and help (b p.182) • d weight to optimum for height (b p.178) • Encourage regular ­exercise—dynamic is best, e.g. walking, swimming, cycling (b p.180) • d alcohol to 3wk for urgentCXRN. • Refer any unexplained lump in the neck of recent onset, or any previously undiagnosed neck lump that has changed over a period of 3–6wk, for urgent further investigationN.

Other signs of respiratory disease

Clubbing  bp.605 • Refer any patient with unexplained nail clubbing for urgentCXRN.

Yellow nails  bp.604 Hoarseness  bp.936 • Refer urgently for chest X-ray (CXR)N  ALL patients with hoarseness for >3wk—particularly smokers aged >50y and heavy drinkers. • If there is a POSITIVE finding on CXR Refer urgently to a team specializing in the management of lung cancer • If there is a NEGATIVE finding on CXR Refer urgently to a team specializing in the management of head and neck cancer Stridor  bp.937 Jugular venous pressure  bp.233 The trachea • Palpate the trachea in the supraclavicular notch in the midline • Deviation to the left or right suggests a shift of the upper mediastinum to thatside • The distance between the suprasternal notch and cricoid cartilage in an adult is 2–3 finger breadths. If it is less than this, the lungs are probably hyperinflated

Further information NICE Referral guidelines for suspected cancer (2005) M www.nice.org.uk

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Respiratory investigations Indications for urgentCXRN

• Haemoptysis • Any of the following if unexplained or present for more>3wk: • Weightloss • Cough • Chestsigns • Chest/shoulderpain • Hoarseness • Dyspnoea • Cervical/supraclavicular lymphadenopathy • Finger clubbing • Signs suggesting metastases (brain, bone, liver,skin) Peak flow  A simple and cheap test. Peak flow is not a good measure of airflow limitation as it tends to overestimate lung function. It is best used to monitor progress of disease and effects of treatment for patients with asthma. Link with self-management plan (b p.310). Peak flow meters are available on NHS. Peak flow charts are available from NHS supplies (form FP1010) and drug companies. Measuring peak expiratory flow rate(PEFR) • Ask the patient to stand up (if possible) and hold the peak flow meter horizontally. Check the indicator is at zero and the trackclear • Ask the patient to take a deep breath in and blow out forcefully into the meter ensuring lips are sealed firmly around the mouthpiece • Read the PEFR off the meter. The best of three attempts is recorded • Consider using a low range meter if predicted or best PEFR is 5y) is able to expel from the lungs after a maximal inspiration (see Table11.3). • FEV1 Volume of air the patient is able to exhale in the first second of forced expiration • FVC Total volume of air the patient can forcibly exhale in one breath • FEV1/FVC Ratio of FEV1 to FVC expressed asa% Measuring FEV1 andFVC  Sit the patient comfortably, then: • Ask the patient to take a deep breathin • Ask the patient to blow the whole breath out as hard as possible until there is no breath left to expel and ensuring lips are sealed firmly around the mouthpiece; encourage the patient to keep breathing out • Repeat the procedure twice (i.e. three attempts inall) • At least two readings should be within 100mL or 5% of eachother • Normal values—see Table11.5 (b p.305) Flow volume measurement  Available with some spirometers—see Figure11.1. Fractional exhaled nitric oxide concentration  Exhaled nitric oxide concentration is i in patients with asthma or other inflammatory respiratory conditions. Measurement of exhaled nitric oxide (usually with hand-held monitor) can be a useful test to support a diagnosis of asthma or assess response to treatment. A normal result does not exclude a diagnosis of asthma. RCP three questions Useful tool to identify patients with poor asthma control in general practice and monitor effect of changes of treatment. Morbidity categories correlate with lung function.

Respiratory investigations

In the lastmonth • Have you had any difficulty sleeping because of your asthma symptoms (including cough)? • Have you had your usual asthma symptoms during the day (cough, wheeze, chest tightness, or breathlessness)? • Has your asthma interfered with your usual activities, e.g. housework, work/schooletc.? NO to all questions = low morbidity. 1x YES answer = medium morbidity. 2 or 3x YES answers = high morbidity. 0 Alternatives include the Asthma Control Questionnaire (ACQ) and Asthma Control Test (ACT)/Children’s Asthma Control Test. These questionnaires are not designed for use during an acute attack.

Further information

NICE Referral guidelines for suspected cancer (2005) M www.nice.org.uk OBSTRUCTIVE PICTURE

RESTRICTIVE PICTURE Normal

i Expiratory flow rate (L/s)

Lung disease

Concave curve FVC d

Volume (L) l

Figure11.1  Flow volume curves for patients with restrictive and obstructive lung

disease

Table11.3  Interpretation of spirometry results Restrictive lung disease, e.g. interstitial lung disease

Obstructive lung disease, e.g. COPD

FEV1 (% of predicted normal)

d(1 x 109/L)—R

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Asthma management in practice Aims of treatment To: • d symptoms and impact on lifestyle (e.g. absence from work/school) • Minimize the need for reliever medication • Prevent severe attacks/exacerbations GP services  Routine asthma care should be carried out in a specialized clinic. Doctors/nurses involved need appropriate training and regular updates. Practices should keep an asthma register to ensure adequate follow-up and allow audit. 0 Not all patients want to attend a pre­ arranged appointment. Telephone reviews may be as effective as face-toface consultations. Reviews and monitoring Frequency depends on needs. Aim to review all patients with asthma at least annually (see Figure11.2). • Check symptoms since last seen. Use objective measures, e.g. RCP three questions (b p.303) • Record smoking status and advise smokers tostop • Record any exacerbations/acute attacks since lastseen • Check medication—use, concordance (prescription count— bp.142), inhaler technique, problems, side effects • Check influenza/pneumococcal vaccination received • Review objective measures of lung function e.g. home PEFR chart, PEFR/spirometry atreview • Address any problems or queries and educate aboutasthma • Agree management goals and date for furtherreview Self-management  All patients should receive: • Self-management education Brief, simple education linked to patient goals is most likely to be successful. Include information about:nature of disease, nature of the treatment and how to use it, self-monitoring/ self-assessment, recognition of acute exacerbations, allergen/trigger avoidance, patients’ own goals of treatment • Written action plan Focus on individual needs. Include information about symptom triggers and peak flow levels that indicate when asthma is worsening, and guidance about what to do under those circ*mstances. Action plans d morbidity and health costs from asthmaS • PEFR monitoring Record PEFR at asthma review and if acute exacerbation. Home monitoring + action plan can be useful, especially for patients with severe asthma, brittle asthma (i.e. rapid development of acute asthma attacks) and/or if poor perceivers of symptoms • Be aware that those from ethnic minorities, socially disadvantaged groups, those with communication problems, adolescents, and the elderly have complexneeds. Management of acute asthma  b pp. 1092–7 Non-pharmacological measures 

• Smoking Smoking may i symptoms of asthma—advise tostop • Weight There is some evidence that weight d in obese patients with asthma results in i asthma controlR

Asthma management in practice

• Allergen avoidance • House dust mite There is little evidence that d house dust mite results in clinical improvementC. In committed families advise:complete barrier bed coverings; removal of carpets; removal of soft toys from bed; high-temperature washing of bed linen; acaricides to soft furnishings; dehumidification. There is no evidence that air ionizers have any beneficial effect • Pets There is no evidence that removing pets from a home results in improved symptoms but many experts still advise removal of the pet for patients with asthma who also have an allergy to thepet Drug therapy  bp.312

Patient information and support Asthma UK F 08457 01 02 03 M www.asthma.org.uk

Symptoms

Include objective measures, e.g. ACQ, ATQ, or RCP three questions (b p. 303)

Smoking status

Record and advise about smoking cessation

Exacerbations

Numbers and circ*mstances

Medication

Use/concordance Problems Side effects Inhaler technique

Examination

Education

Objective measures of lung function, e.g. PEFR, spirometry Tailor to the individual: Treatment Monitoring Allergen avoidance

Goals and next review

Figure11.2  Summary of the annual asthmareview

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Drug treatment ofasthma Management of acute asthma  b pp. 1092–7 Use a stepwise approachG  (see Figure11.3). Start at the step most

appropriate to the initial severity of symptoms. The aim is to achieve early control of the condition and then to d treatment by steppingdown.

Exacerbations Treat early. In adult patients on 200 microgram doses of inhaled steroids, a 5x i in dose reduces severity of exacerbations. Alternatively, and in all other cases, use prednisolone 30–40mg od for1–2wk. Selection of inhaler device  If possible use an MDI. Inadequate technique may result in drug failure. Patients must inhale slowly and hold their breath for 10s after inhalation. Demonstrate inhaler technique before prescribing and check at follow-ups. Spacers/breath-activated devices are useful if patients find activation difficult. Dry powder inhalers are an alternative. Short-acting β2 agonists  b p.306 (e.g. salbutamol). Work more quickly and with fewer side effects than alternatives. Use prn unless shown to benefit from regular dosing. Using �2 canisters/mo or >10–12 puffs/d is a marker of poorly controlled asthma. 0 Abudesonide/formoterol combination inhaler is an alternative rescue medication. Inhaled corticosteroids  (b p.306) Effective preventer. May be beneficial even for patients with mild asthma. Consider if:exacerbation of asthma in the last 2y requiring steroids; using inhaled β2 agonists �3x/wk; or symptomatic � 3x/wk or �1 night/wk. Oral steroids  bp.306 Add-on therapy Aims to improve lung function/symptoms. Before initiating a new drug, check compliance, inhaler technique, and eliminate trigger factors. Only continue if of demonstrable benefit. • Inhaled long-acting β2 agonists (LABA) b p.306 (e.g. salmeterol). Do not use without inhaled steroids. Combination inhalers (steroid + LABA) improve compliance • Leukotriene receptor agonists (e.g. montelukast) d exacerbations • Theophylline Side effects are common Stepping down  Review and consider stepping down at intervals �3mo. Maintain on the lowest dose of inhaled steroid controlling symptoms. When reducing steroids, cut dose by 25–50% eachtime. Omalizumab  Monoclonal antibody that binds to circulating IgE. Useful for adults and children >6y if allergy is a factor in asthma, on high-dose inhaled steroid + LABA, and frequent exacerbations. Given sc every 2–4wk. Always specialist-initiated. Complementary therapies  Buteyko breathing technique d symptoms/bronchodilator use. Immunotherapy in specialist clinics is effective for patients with specific allergies. Other complementary therapies—no convincing evidence of effectiveness.

Drug treatment ofasthma

* All doses given refer to hydrofluoroalkane-beclometasone dipropionate (BDP-HFA) equivalent inhalers. For other drugs/formulations adjust dose accordingly (see BNF Section3).

Figure11.3  Summary of stepwise management inadults Reproduced from British Guideline on the management of asthma (May 2008, rev. Jan 2012), p48 with permission from the Scottish Intercollegiate Guidelines Network.

Difficult asthma  Persistent symptoms and/or frequent exacerbations despite treatment at step 4/5. Check diagnosis and exacerbating factors. Assess adherence to medication. Find out about family, psychological, or social problems that may be interfering with effective management.

Further information British Thoracic Society/SIGN British guideline on the management of asthma (2011) M www.sign.ac.uk BNF Section 3 M www.bnf.org

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Chronic obstructive pulmonary disease Chronic obstructive pulmonary disease (COPD) is a slowly progressive disorder characterized by airflow obstruction. Affects 716% of the population in the 40–68y age group (73million people in the UK—two-thirds undiagnosed). 4 > 5. Responsible for 75% of deaths.

Causes 

• Cigarette smoking • Genetic—bronchial hyperresponsiveness; α1-antitrypsin deficiency • Race—Chinese and Afro-Caribbeans have d susceptibility • Diet—poor diet and low birthweight

Diagnosis  Is suggested by history, signs, and baseline spirometry. Consider in any patient >35y with a risk factor for COPD (generally smoking) and �1of: • Shortness of breath on exertion—use an objective measure, e.g. MRC dyspnoea scale (see Table11.6) to grade breathlessness • Chroniccough • Regular sputum production • Wheeze • Frequent winter ‘bronchitis’ Table11.6  MRC dyspnoeascale Grade

Degree of breathlessness related to physical activity

1

Not troubled by breathlessness, except on strenuous exercise

2

Short of breath when hurrying or walking up a slight hill

3

Walks slower than contemporaries on level ground because of breathlessness or has to stop for breath when walking at own pace

4

Stops for breath after walking 100m or after a few minutes on level ground

5

Too breathless to leave the house or breathless on dressing/undressing

If diagnosis is suspected also ask about  weight d, effort intolerance, waking at night, ankle swelling, fatigue, and occupational hazards. • Chest pain or haemoptysis are uncommon in COPD—if present consider an alternative diagnosis. Signs  May be none. Possiblesigns: • Hyperinflated chest ± poor chest expansion on inspiration • d cricosternal distance • Hyperresonant chest with d cardiac dullness on percussion • Wheeze or quiet breathsounds • Paradoxical movement of lowerribs

• Use of accessory muscles • Tachypnoea • Pursing of lips on expiration (purse lip breathing) • Peripheraloedema • Cyanosis • iJVP • Cachexia

Spirometry  (b p.302) Predicts severity (see Table11.7) and prognosis but not disability/quality of life. Diagnose airflow obstructionif: • FEV1/FVC 4. Non-respiratory manifestations of sarcoidosis • Heart failure • Fever and malaise • Pericardial effusion • Erythema nodosum • Cranial and/or peripheral nerve • Lupus pernio (blue red nodules palsies on the nose, face, and/orhands) • Seizures • Scar infiltration • Hypercalcaemia • Enlarged lacrimalglands • Renalstones • Hypopyon • Lymphadenopathy • Uveitis • Hepatosplenomegaly • Arthralgia • Arrhythmias Acute sarcoidosis (Löfgren syndrome) • Polyarthralgia • Erythema nodosum • Swingingfever • Bilateral hilar lymphadenopathyonCXR

Interstitial lung disease

Insidious onset  CXR shows hilar lymphadenopathy—incidental finding in 30–50%. If symptomatic, usually presents with tiredness, malaise, weight d and/or arthralgia. 15% have lung symptoms with gradual onset of progressive exertional dyspnoea and drycough. Management  Refer any patient with bilateral hilar lymphadenopathy for further investigation. For patients with confirmed sarcoidosis, specialist management is needed. Steroids are the first-line treatment but should only be usedif: • Progressive disease (on imaging or lung function testing) • Significant symptoms,or • Extrapulmonary disease requiring treatment Rarely, if steroids are not controlling disease progression or symptoms, methotrexate will be added. Inhaled steroids may be helpful to control cough but do not influence disease progression. For patients with severe symptoms, pulmonary rehabilitation may be helpful. Lung transplant may be considered for patients with end-stage pulmonary sarcoidosis. Prognosis  Remits without treatment in 2:3 cases. Acute sarcoidosis has good prognosis with most resolving in 16"), nasal congestion, evening alcohol/sedatives, large tonsils, receding lower jaw, smoking, hypothyroidism, menopause.

Management Snoring without sleep apnoea  • Initial approaches Suggest changing sleeping position (discourage from sleeping on back); elevate head of the bed (e.g. prop up on bricks—can d nasal congestion); limit number of pillows to one thick/ two thin pillows to maximize pharyngeal size; d weight if obese; dor stop evening alcohol/sleeping tablets; suggest partner tries ear plugs (purchase from chemist—takes several nights to get used to wearingthem) • If clinically indicated • Nasal congestion—start beclometasone nasal spray (applied head downwards) 2 puffs bd ± ipratropium bromide nasal spray 2 puffsnocte • Check TFTs to exclude hypothyroidism • Discuss the use of HRT in menopausal women • If simple measures fail Referto: • Dentist or ENT for a mandibular advancementdevice • ENT for surgery—septal straightening, polypectomy, turbinate reduction, tonsillectomy, or uvulopalatopharyngoplasty

Snoring and obstructive sleepapnoea

Sleepapnoea • Advise patients to:d weight if obese; d or stop evening alcohol/ sleeping tablets,and • Refer to a sleep unit or physician with a special interest in sleep problems. If diagnosis is proven and causing significant daytime sleepiness, usual treatment is with CPAP therapy at night. Mandibular advancement devices are alternatives for patients who cannot tolerate CPAP or have very mild symptoms with no daytime sleepiness. Occasionally, if large tonsils, referral to ENT for surgery is warranted

• Driving  Warn patients NOT to drive if sleepy. Once diagnosis of sleep

apnoea is confirmed, they must inform the DVLA and their insurance company (b p.130).

Sleep apnoea in children Common in children aged 2–7y in association with tonsil enlargement during URTI. Sleep disruption can cause daytime sleepiness, hyperactivity, poor attention span, and bad behaviour. If tonsils are big enough to produce sleep apnoea in the absence of current infection, refer to ENT for consideration of tonsillectomy. How likely are you to doze off or fall asleep in the following situations, in contrast to feeling just tired? This refers to your usual way of life in recent times. Even if you have not done some of these things recently try to work out how they would have affected you. Situation

Chance of dozing

Sitting and reading

Watching TV

Sitting inactive in a public place (e.g. a theatre or a meeting)

As a passenger in a car for an hour without a break

Lying down to rest in the afternoon when circ*mstances permit

Sitting and talking to someone

Sitting quietly after a lunch without alcohol

In a car, while stopped for a few minutes in traffic 0=no chance of dozing 1=slight chance of dozing

 2=moderate chance of dozing 3=high chance of dozing

If score > 10 – consider sleep apnoea

Figure 11.7  The Epworth Sleepiness Scale Reproduced from: Johns MW (1991) A new method for measuring daytime sleepiness: the Epworth Sleepiness Scale. Sleep 14:540–5, with permission from Associated Professional Sleep Societies, LLC.

Further information SIGN/British Thoracic Society Management of obstructive sleep apnoea/ hypopnoea syndrome in adults (2003) M www.sign.ac.uk

Patient support The Sleep Apnoea Trust (SATA) M www.sleep-apnoea-trust.org

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Endocrinology Symptoms of endocrine disease  342 Diabetes mellitus  344 Organization and monitoring of care  346 Management of diabetes: education  348 Treatment of type 2 diabetes  350 Treatment with insulin  352 Diabetic complications:cardiovascular  354 Diabetic complications:renal and eye  356 Diabetic complications:nerve and skin  358 The diabetic foot  360 Lumps in the thyroid gland and goitres  362 Thyroid disease  364 Hyper- and hypocalcaemia  366 Adrenal disorders  368 Pituitary problems  370

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Symptoms of endocrine disease Hormones secreted by the endocrine system perform a wide range of functions. Therefore, clinical presentation of different endocrine disorders varies widely from non-specific symptoms such as tiredness, to very specific signs such as delayed puberty. Specific features depend on the gland and hormones involved.

Polydipsia Over-frequent drinking of fluid—often associated, for logical reasons, with polyuria. Ask if associated with thirst. Take a history of fluid intake. If no history of excess fluid intake and BM/fasting blood glucose/ HbA1c is normal, investigate further with U&E, Cr, andCa2+. Commoncauses • Change in lifestyle:diet/activity/exercise level—may be associated with polyuria but no other symptoms. No history of thirst • DM—usually accompanied by a history of thirst Othercauses Diarrhoea, diabetes insipidus, i Ca2+, compulsive water drinking (may be a feature of psychotic illness), phosphorus poisoning.

Polyuria Passage of excessive urine. Check the patient does not mean

frequency of urination. It can be difficult to distinguish the two. Causes are similar to those of polydipsia and the 2 symptoms are related. Take a history of fluid intake. If no history of excess fluid intake and BM/fasting blood glucose/ HbA1c is normal, investigate further with MSU (for M,C&S), U&E, Cr, andCa2+. Consider • DM—always check a BM and/or fasting blood glucose if a patient complains of polyuria • Diabetes insipidus • Hypercalcaemia • Excessive intake—due to change in lifestyle or psychiatric conditions, e.g. schizophrenia • Chronic renal failure • Drugs—diuretics, caffeine, alcohol

Glycosuria Often detected incidentally on urine dipstick. Causes: • DM • Sepsis •  Low renal threshold • Pregnancy • Renal tubular damage In all cases, check HbA1c/fasting blood glucose (+ glucose tolerance test if pregnant). Check immediate BM if other symptoms suggestiveofDM.

Hirsutism Affects 10% 5. Excess hair in androgenic distribution. Causes: • Most cases are idiopathic; there may be a family history • Drugs—phenytoin; corticosteroids; ciclosporin; androgenic oral contraception; anabolic steroids; minoxidil; diazoxide • Polycystic ovarian syndrome (PCOS) • Cushing’s syndrome • Late-onset congenital adrenal •  Ovarian tumours (rare) hyperplasia (rare)

Symptoms of endocrine disease

Assessment • History Long-standing or recent onset, family history, ethnic origin (more common in Mediterranean countries), menstrual history • Examination Distribution of excesshair Investigation Women with longstanding hirsutism (since puberty) and regular periods need no further investigation unless abnormalsigns. Otherwise: blood—testosterone (i in PCOS, androgen-secreting tumour, late-onset congenital adrenal hyperplasia); LH/FSH ratio (>3:1 suggestsPCOS). Refer to gynaecologist or endocrinologistif recent onset, abnormal blood tests, virilism, galactorrhoea, menstrual disturbance, infertility, and/or pelvicmass. Treatment of idiopathic hirsutism • Cosmetic—bleaching, shaving, waxing, depilatory creams, electrolysis • Weight d in obese individuals • Psychological support • Topical eflornithine d growth of unwanted facial hair. Continuous use for >8wk is required before benefit is seen. Must be used indefinitely to prevent regrowth. Discontinue if no improvement in4mo • Oral medication—all must be taken for ≥6mo to take effect and none abolishes the problem. In all cases, continue treatment until acceptable level of hair growth then stop. Relapse usually follows withdrawal and repeat courses are then required. Drugs used: COC pill containing desogestrel or co-cyprindiol; spironolactone

Menopause  b p. 710 Delayed or precocious puberty  bp.893 Obesity  bp.178 Metabolic syndrome (syndrome X; insulin resistance syndrome) Impaired glucose tolerance or DM, insulin resistance (in

patients on insulin, suggested by insulin doses >1u/kg/d) + other risk factors for CVD including: • Truncal obesity—waist circumference >0.9m (5); >1.0m (4), use 0.1m lower figures for people of south Asian extraction • i BP—>135/80 • Dyslipidaemia—serum HDL 65 • Ethnic group—South Asians/Afro• Obesity Caribbeans have 5–10x irisk • FH of DM (identical twin •  PMH of gestational diabetes or a baby concordance 8100%) >4kg atbirth • Impaired glucose tolerance Progressive disease resulting from impaired insulin secretion and insulin resistance. Life expectancy is d by 30–40% in the age range 40–70y—a d of 8–10y of life. Onset is often insidious; 50% have complications at diagnosis. Latent autoimmune diabetes in adulthood(LADA) 6–10% of patients with type 2 DM. Characterized by anti-glutamic acid decarboxylase (GAD) antibodies. Associated with higher risk of ketoacidosis and i risk of progression to insulin dependence. Suspect if type 2 DMand: • Absence of metabolic syndrome features • Uncontrolled hyperglycaemia despite oral agents,and/or • Other autoimmune diseases (e.g. thyroid disease, pernicious anaemia) Maturity onset diabetes of the young(MODY) 1–2% of patients with DM. Present 48mmol/mol DM is likely.

Diabetes mellitus

Presentation • Acute Ketoacidosis or hyperosmolar non-ketotic coma (b p.1100) • Subacute Weight d, polydipsia, polyuria, lethargy, irritability, infections (candidiasis, skin infection, recurrent infections slow to clear), genital itching, blurred vision, tingling in hands/feet • With complications Skin changes, neuropathy, nephropathy, arterial or eye disease (b pp.354–61) • Asymptomatic Incidental finding or through risk stratification

Risk stratificationN Use a risk stratification tool (e.g. Diabetes Risk Score or QDiabetes) to assess all patients: • >40y,or • >25y of South Asian, Chinese, Afro-Caribbean, or black African origin, from hard-to-reach populations (e.g. homeless) or with other medical conditions that predispose to DM (e.g. pancreatitis) If low/intermediate risk, give lifestyle advice and reassess in 5y. If high risk or of South Asian/Chinese ethnic origin and BMI >23kg/m2, check FBG orHbA1c: • FBG 4d prior to treatment, and do not restart until >3d after. Advise women of childbearing age to avoid pregnancy for 4mo. Most become hypothyroid at some point (sometimes years) after treatment. Continue monitoring TFTs long-term. Associated with small i risk of thyroid malignancy • Surgery Partial or total thyroidectomy—reserved for patients with large goitres or who decline radioactive iodine. Carries risk of damage to recurrent laryngeal nerve or parathyroids • Warn all patients starting carbimazole to stop the drug and seek urgent medical attention if they develop sore throat or other infection.

Thyrotoxic crisis/storm b p.1101 Graves’ disease Most common cause of hyperthyroidism. 5:48 5:1.

Peak age: 30–50y. Associated with smoking and stressful life events. Autoimmune disease in which antibodies to the TSH receptor are produced. R.J. Graves (1797–1853)—Irish physician. Clinical features • Hyperthyroidism • Diffuse goitre ± thyroid bruit due to i vascularity • Extrathyroid features:thyroid eye disease—25–50% (bilateral in >90%); pretibial myxoedema—5%; thyroid acropachy (clubbing, finger swelling)—rare; onycholysis—rare Management As for hyperthyroidism.

Thyroid disease

Table12.5  Interpretation of thyroid function test results Results of TFTs

Interpretation

Notes

TSH d, T4i

Thyrotoxic

Occasionally T4 is normal but T3i

TSH i, T4d

Hypothyroid

TSH d if hypothyroidism is secondary to pituitary failure (rare)

TSH i, T4n

Subclinical hypothyroidism

If any symptoms (including depression and non-specific symptoms or hyper­ cholesterolaemia) consider a trial of treatment If no symptoms monitor annually

Thyroid eye disease Presentswith:

• Ophthalmoplegia (especially of • Double vision upwardgaze) • Eye discomfort ± protrusion • TFTs can be i or normal (exophthalmos and proptosis) • Lidlag Management  Refer to ophthalmologist. If d acuity or loss of colour vision—refer urgently as there may be optic nerve compression. Hypothyroidism (myxoedema) Common—10% 5 >60y, 5:488:1. Causes Chronic autoimmune thyroiditis, post-131I, thyroidectomy. Presentation Onset tends to be insidious and may go undiagnosed for years. Always consider hypothyroidism when a patient has non-specific symptoms, depression, fatigue, lethargy, or general malaise. Other symptoms—weight i, constipation, hoarse voice, or dry skin/hair. Signs are often absent—there may be a goitre, slow-relaxing reflexes, or non-pitting oedema of the hands, feet, or eyelids. Screening Check TFTs in patients: • With persistent symptoms of tiredness/lethargy without clearcause • On amiodarone or with a history of 131I administration • With hypercholesterolaemia, infertility, Turner’s or Down’s syndrome, depression, dementia, obesity, DM, or other autoimmune disease Management Patients taking thyroxine replacement are entitled to apply for free prescriptions in England (b p.137). • 3.5mmol/L or severe symptoms, admit for lowering of Ca2+ with forced diuresis and IV bisphosphonate.

Hyper- and hypocalcaemia

Hyperparathyroidism i secretion of parathyroid hormone(PTH). • p hyperparathyroidism Incidence 0.5/1,000. Peak age 40–60y. 5:48 2:1. Circulating level of PTH is inappropriately high. Most patients are hypercalcaemic (but may be normocalcaemic if coexistent vitamin D deficiency). Due to i secretion of PTH from one or both parathyroid glands. Refer. Treatment is usually surgical • s hyperparathyroidism i PTH in response to chronic hypocalcaemia or hyperphosphataemia. Treatcause • Tertiary hyperparathyroidism Inappropriately i PTH li Ca2+. Follows prolonged s hyperparathyroidism. Most common in patients with chronic renal failure (especially if on dialysis) or chronic malabsorption. Treatment is usually surgical

Familial benign hypercalcaemia Asymptomatic. Inherited condition in which serum calcium concentrations are mildly i throughout life. Confirm (if possible) by demonstrating i Ca2+ in other family members. No adverse consequences and no treatment needed. Milk alkali syndrome Usually due to ingestion of OTC indigestion remedies (e.g. Rennies®). Ca2+ levels revert to normal on stopping. Investigate the reason why the patient is taking these remedies (? peptic ulcer). Sometimes also caused by calcium supplements taken with bisphosphonates for prophylaxis of osteoporosis—stop the calcium supplement.

Hypercalcaemia Albumin i Urea i

Urea normal

Dehydration— Rehydrate and recheck Cuffed specimen— Repeat uncuffed

Albumin normal or d Phosphate d Phosphate i or normal or normal Urea normal

Primary or tertiary hyperparathyroidism Alk phos i Bone metastases Sarcoidosis Thyrotoxicosis

Alk phos normal Myeloma Vitamin D excess Milk alkali syndrome

Figure12.3  Guide to the diagnosis of cause of hypercalcaemia (must be taken in clinical context)

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Adrenal disorders Disorders of the adrenalcortex The adrenal cortex produces three classes of steroids: • Glucocorticoids, e.g. cortisol • Mineralocorticoids, e.g. aldosterone,and • Sex hormones, e.g. androstenedione, testosterone, and oestrogen Symptoms result from disturbance in production of these steroids. Cushing’s syndrome In the majority of cases, Cushing’s syndrome is iatrogenic—caused by exogenous administration of prednisolone or other corticosteroids. Non-iatrogenic Cushing’s syndrome is much rarer with annual incidence of 1–2/million (5:4 83:1): • 80% have a pituitary adenoma which secretes adrenocorticotrophic hormone (ACTH) causing hypersecretion of glucocorticoids and sex hormones (Cushing’s disease) • 20% are due to ectopic ACTH secretion by other tumours (e.g. small cell lung cancer) or hypersecreting tumours of the adrenal cortex H.W. Cushing (1869–1939)—US neurosurgeon. Presentation Cushing’s syndrome has high morbidity and mortality. Clinical features include: • Osteoporosis(60%) • Pigmentation • Moon face(90%) • Feminization inmen • Truncal obesity(85%) • Lethargy/ • Polyuria and depression(60%) •  Hypertension(80%) polydipsia • Hirsuitism • Menstrual • Psychosis • Acne disturbance(80%) • Striae and bruising(60%) Management • Stop/minimize exogenous steroids • If no exogenous steroids and Cushing’s syndrome is suspected, request a dexamethasone suppression test—dexamethasone 1mg po at midnight, then serum cortisol measured at 9 a.m. If 600nmol/L—adrenal insufficiency is excluded • 400–590nmol/L—the result is equivocal; repeat • 1). Specialist investigations (e.g. water deprivation test) confirm diagnosis. Management  Treat thecause. • Cranial DI may be treated with intranasal desmopressin or surgery • Nephrogenic DI may be treated with dietary restriction of protein and salt and/or bendroflumethiazide

Syndrome of inappropriate ADH (SIADH)  Important cause of

hyponatraemia. Diagnosis is made by finding a concentrated urine (sodium >20mmol/L) in the presence of hyponatraemia (4wk or any atypical features, consider referral for urgent investigation—b p.406. Gastroenteritis  bp.410

Chronic diarrhoea and malabsorption  bp.406 Faecal incontinence  bp.408 Melaena or rectal bleeding  b p.1076 Factitious diarrhoea  bp.407 • Some children may become cow’s milk intolerant after a bout of gastroenteritis—bp.889 • Think of haemolytic uraemic syndrome in any child with diarrhoea who passes blood in thestool

Further information NICE Diarrhoea and vomiting in children under 5 (2009) M www.nice.org.uk

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Constipation 3 million GP consultations/y in the UK result from constipation. Differentiate normal stools a few days apart (normal, needs no treatment) and infrequent hard stools (suggests constipation). Definition  Two or more of the following for≥3mo: • Straining at defecation ≥25% of thetime • Asensation of incomplete evacuation ≥25% of thetime • ≤2 bowel movements/wk • Lumpy and/or hard stools ≥25% of thetime 0 Most patients consulting in general practice do not meet these criteria. Children with constipation  bp.888 Young patients 6wk • Treat any reversible, underlying organic cause—see Table13.3 • Give lifestyle advice (see management of young people with lone constipation) • Treat symptomatically if no cause is found/cause is untreatable • Laxatives—consider an osmotic (e.g. lactulose, magnesium hydroxide) or bulk-forming laxative (e.g. ispaghula, sterculia) ± a stimulant laxative (e.g. senna). Titrate dose to response • Long-term use of stimulant laxatives including co-danthrusate is acceptable in the very elderly. Otherwise, use prn or intermittently • If oral laxatives are ineffective consider adding rectal measures. If soft stool, try bisacodyl suppositories (0 must come into direct contact with rectum); if hard stools try glycerin suppositories (act in1–6h) • If still not cleared/faecal impaction—refer to the district nurse for lubricant ± high phosphate (stimulant) enema (acts in 720min) • Once constipation has been cleared, leave the patient with clear instructions about what to do if symptomsrecur

• High-risk patients,  e.g. patients on opioids; those who are immobile or have medical conditions which predispose them to constipation. Pre-empt constipation by putting high-risk patients on regular aperients. Occult presentations of constipation  are common in the elderly and include: • Overflow diarrhoea • Confusion • Loss of appetite andnausea • Urinary retention • Abdominalpain

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Other abdominal symptoms andsigns Dyspepsia  bp.382 Abdominal distension  Consider abdominal/pelvic massesand: • Fluid—ascites or full bladder • Fat

• Faeces • Fetus • Flatus—intestinal • Food, e.g. obstruction; air swallowing malabsorption Abdominal masses Distinguished from pelvic masses by the ability to get beneath them. Causes: Malignancy—any intra-abdominal organ or kidney; stool; abdominal aortic aneurysm; hepato- and/or splenomegaly; appendix mass/abscess; Crohn’s mass; lymph nodes or TB mass. 0 Ahernia may present as a mass in abdominal wall/groin lump—b p.392. Pelvic masses Causes: fetus; full bladder; fibroids; gynaecological malignancy; bladder cancer.

Splenomegaly  Causes: • Haematological Lymphoma, leukaemia, myeloproliferative disorders, sickle cell disease (children usually), thalassaemia • Inflammatory RA or Sjögren’s syndrome, sarcoid, amyloid • Infection Glandular fever, malaria, SBE, TB, leishmaniasis Hepatomegaly  Causes: • Apparent Reidel’s lobe, low-lying diaphragm • Tumours Secondary (most common), primary • Venous congestion Heart failure, hepatic vein occlusion • Haematological Leukaemia, lymphoma, myeloproliferative disorders, sickle cell disease • Biliary obstruction Particularly extrahepatic • Inflammation Hepatitis, abscess, schistosomiasis • Metabolic Fatty liver, amyloid, glycogen storage disease • Cysts Polycystic liver, hydatid Ascites  Free fluid in the peritoneal cavity. Signs: abdominal distension, shifting dullness to percussion, fluid thrill. Causes: Malignancy—any intra-abdominal organ, ovary, or kidney; hypoproteinaemia, e.g. nephrotic syndrome; right heart failure; portal hypertension. Fistula  Abnormal communication between one organ and another— usually due to cancer or complication of surgery. Presentation— Table13.4. Refer urgently if suspected. Table13.4  Presentation of fistula Connection

Presentation

Bowel l skin

Faecal discharge through surgical wound

Bladder/ureters l skin

Clear, watery discharge which smells of urine

Bowel l vagin*

Faeculent material in vagin*

Bladder l vagin*

Leakage of urine per vaginum

Bowel l bladder

Air or faeculent material in urine; recurrent UTI

Other abdominal symptoms andsigns

Urgent referral for upper GI symptomsN  Consider checking FBC when referring, depending on local protocols. Urgent referral to a team specializing in upper GI malignancy  Patients presentingwith: • Dysphagia • Unexplained upper abdominal pain and weight d ± backpain • Upper abdominal mass without dyspepsia • Obstructive jaundice (depending on clinical state)—consider urgent USS if available Consider urgent referral to a specialist in upper GI malignancy  Patients presentingwith: • Persistent vomiting and weight d in the absence of dyspepsia • Unexplained weight d or iron deficiency in the absence of dyspepsia • Unexplained worsening of dyspepsia and Barrett’s oesophagus; known dysplasia, atrophic gastritis, or intestinal metaplasia; or peptic ulcer surgery >20yago Consider urgent specialist referral or referral for urgent endoscopy  Patients of any age with dyspepsiaand: • Persistent vomiting • Chronic GI bleeding • Iron deficiency anaemia • Dysphagia • Epigastricmass • Progressive unintentional • Suspicious barium mealresult weightd Urgent referral for endoscopy  Any patient ≥55y and with unexplained (i.e. no obvious cause, e.g NSAIDs) and persistent, recent-onset dyspepsia alone. GPs should not allow symptoms to persist >4–6wk before referral. 0 Helicobacter pylori status should not affect the decision to refer for suspected cancer. Consider checking FBC to exclude iron deficiency anaemia in all patients presenting with new-onset dyspepsia. Urgent referral for lower GI symptomsN  Refer urgently to a team specializing in lower GI malignancyif: Any age  with: • Right lower abdominal mass consistent with involvement of largebowel • Apalpable rectal mass (intraluminal, not pelvic; a pelvic mass outside the bowel would warrant an urgent referral to a urologist) • Unexplained iron deficiency anaemia (Hb ≤110g/dL for 4 ≤100g/dL for a non-menstruating5) Aged ≥40y  Reporting rectal bleeding with a change of bowel habit towards looser stools and/or increased stool frequency persisting≥6wk. Aged ≥60y  with: • Rectal bleeding persisting for ≥6wk without a change in bowel habit and without anal symptoms • Change in bowel habit to looser stools and/or more frequent stools persisting for ≥6wk without rectal bleeding 0 In a patient with equivocal symptoms who is not unduly anxious, it is reasonable to ‘treat, watch, andwait’.

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Dyspepsia and H.pylori In any year, up to 40% of the adult population suffer from dyspepsia—1:10 seek their GP’s advice; 710% of these are referred for endoscopy.

Causes • Gastro-oesophageal reflux disease (GORD)—15–25% (b p.386) • Peptic ulcer (PU)—15–25% (b p.388) • Stomach cancer—2% (b p.390) • The remaining 60% are classified as non-ulcer dyspepsia (NUD, ‘functional’ dyspepsia)—manage as for uninvestigated dyspepsia • Rarer causes: oesophagitis from swallowed corrosives, oesophageal infection (especially in the immunocompromised) Differential diagnosis  Cardiac pain (difficult to distinguish), gallstone pain, pancreatitis, bile reflux.

Presentation  Common symptoms include retrosternal or epigastric pain, fullness, bloating, wind, heartburn, nausea, and vomiting. Examination is usually normal though there may be epigastric tenderness. Check for clinical anaemia, epigastric mass/hepatomegaly, and LNs in theneck. Management  See Figure13.2. Helicobacter pylori  Infection is associatedwith: • GI disease—peptic ulcer disease; gastric cancer; non-ulcer dyspepsia; oesophagitis • Non-GI disease—ranging from cardiovascular disease and haematological malignancy to cotdeath Testing for H.pyloriN  ‘Test and treat’ all patients with dyspepsia who do not meet referral criteria (see Figure13.2). In practice choice of test is limited by availability, ease of access, and cost. Options in the community are:serology, urea breath test, and faecal antigen test. A2wk washout period following proton pump inhibitor (PPI) use is necessary before testing for H.pylori with a breath test or a stool antigentest. EradicationN  Clears 80–85% H.pylori infections. Options: • PAC500 regimen Full-dose PPI (e.g. omeprazole 20mg bd) + amoxicillin 1g bd + clarithromycin 500mg bd for 1wk,or • PMC250 regimen Full-dose PPI (e.g. omeprazole 20mg bd) + metronidazole 400mg bd + clarithromycin 250mg bd for1wk 0 Do not re-test even if dyspepsia remains unless there is a strong clinical need. Re-test if needed using a urea breathtest. Lifestyle advice  Give advice on healthy eating, weight d, and smoking cessation. Advise patients to avoid precipitating factors, e.g. alcohol, coffee, chocolate, fatty foods. Raising the head of the bed and having a main meal well before going to bed may help some people. Promote continued use of antacids/alginates.

Further information  NICE Management of dyspepsia in adults in primary care (2004) M www.nice.org.uk

Dyspepsia and H.pylori

New Newepisode episodeof ofdyspepsia dyspepsia

Acute Acute GI GI bleeding? bleeding? No

• Chronic gastrointestinal bleeding • Progressive unintentional weight loss • Progressive difficulty swallowing • Persistent vomiting • Iron deficiency anaemia • Epigastric mass • Suspicious barium meal? or • Age >55y and recent-onset, persistent, unexplained dyspepsia— DO NOT delay >4–6wk before referral No • Previous gastric ulcer or surgery • Continuing need for NSAID treatment • i risk of gastric cancer or • Anxiety about cancer

Yes

Admit Yes—urgent referral (4.

Presentation  Gradual onset of dysphagia over years accompanied by regurgitation of stagnant food and foul belching. Night-time coughing fits are due to aspiration which can result in recurrent chest infections. Examination is usually normal although there may be signs of aspiration pneumonia. Management  CXR to exclude aspiration pneumonia; endoscopy confirms diagnosis. Refer for surgery.

Plummer–Vinson syndrome  Iron deficiency anaemia + dysphagia due

to a post-cricoid web in the oesophagus. 5 > 4. Peak incidence: 40–50y. Presents with high dysphagia with food sticking in the back of the throat ± retching/choking sensation. This is a pre-malignant condition so refer for biopsy and dilatation of pharyngeal web; replaceiron. H.S. Plummer (1874–1936); P.P. Vinson (1890–1959)—US physicians.

Oesophageal conditions

Pharyngeal pouch Pulsion diverticulum of the pharyngeal mucosa

through Killian’s dehiscence (area of weakness between the 2 parts of the inferior pharyngeal constrictor). 4 > 5; i with age. Usually develops posteriorly then protrudes to one side—L > R.As the pouch gets larger the oesophagus is displaced laterally. Presentation  Dysphagia—the first mouthful is swallowed easily, then fills the pouch which makes further swallowing difficult. Accompanied by regurgitation of food from the pouch ± symptoms of aspiration (night-time coughing, recurrent chest infection). Aswelling is palpable in the neck in two-thirds ofcases. Management  Refer for further investigation. Diagnosis is confirmed with endoscopy/barium swallow. Treatment is surgical.

Oesophageal varices  Result from portal hypertenion (b p.425) and can bleed massively—admit as a ‘blue light’ emergency if bleeding. Impacted oesophageal foreign body  Usually the patient notices

something has stuck resulting in pain, difficulty swallowing ± retching. If suspected refer immediately to A&E for further investigation ± removal of the foreignbody.

Oesophageal perforation  Rare—usually a complication of endoscopy. Less commonly due to violent vomiting. The patient becomes very distressed with pain relating to the site of perforation which is worse on swallowing. Examination reveals tachycardia, shock ± pyrexia ± breathlessness ± surgical emphysema in neck. Admit as a surgical emergency. Oesophageal atresia and/or tracheo-oesophageal fistula  1:2,500 live births. 5% have oesophageal atresia

alone; 5% tracheo-oesophageal fistula (TOF) alone; the remainder have both. Risk factor for sudden infant death syndrome.

Presentation  • Antenatal:at routine USS or following investigation of polyhydramnios • Post-natal: cough or breathing difficulties in a newborn infant, choking on the first feed, inability to swallow saliva l bubbling of fluid from the mouth, developing soon afterbirth • Later in childhood: ‘H type’ fistulas where there is no atresia, but just a fistula may present late with recurrent chest infections Management  Diagnosis is confirmed with X-ray. Treatment is surgical. Post-operatively children may have a barking cough (‘TOF cough’ ) and/ or dysphagia—both settle before2y.

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Gastro-oesophageal reflux andgastritis Gastro-oesophageal reflux disease (GORD)  Caused by retrograde flow of gastric contents through an incompetent gastro-oesophageal junction. It affects 75% of the adult population. Risk factors • Obesity • Drugs (NSAIDs, TCAs, • Smoking • Hiatushernia SSRIs, iron supplements, • Alcohol • Tight clothes anticholinergics, nitrates, • Coffee • Pregnancy alendronic acid) • Fattyfood • Systemic sclerosis • Surgery for achalasia • Bigmeals Conditions caused byGORD • Oesophagitis (defined by mucosal breaks) ± oesophagealulcer • Benign oesophageal stricture—bp.384 • Intestinal metaplasia:Barrett’s oesophagus • Oesophageal haemorrhage • Anaemia Presentation • Heartburn: most common symptom. Burning retrosternal or epigastric pain which worsens on bending, stooping or lying, and with hot drinks. Relieved by antacids • Other symptoms: • Waterbrash—mouth fills withsaliva • Reflux of acid into the mouth—especially on lyingflat • Nausea and vomiting • Nocturnal cough/wheeze due to aspiration of refluxed stomach contents • Examination: usually normal. Check for clinical anaemia, epigastric mass/hepatomegaly, and LNs in theneck Investigation  Endoscopy if indicated—see Figure13.2, b p.383. 0 Symptoms are poorly correlated with endoscopic findings. Reflux may remain silent in patients with Barrett’s oesophagus but heartburn can severely affect quality of life of patients with–ve endoscopy results. Initial managementN • In all cases, give lifestyle advice (b p.382) • If diagnosis is clinical (i.e. patient presents with ‘reflux-like’ symptoms), treat as for uninvestigated dyspepsia (see Figure13.2, b p.383) • For patients with reflux confirmed on endoscopy, offer treatment with a PPI (e.g. omeprazole 20mg od) for 1–2mo. If oesophagitis at endoscopy and the patient remains symptomatic on PPI, double the dose of PPI for a further1mo • If inadequate response to PPI, try an H2 receptor antagonist (e.g.ranitidine 150mg bd) and/or add a prokinetic (e.g. domperidone 10mgtds) for1mo

Gastro-oesophageal reflux andgastritis

Long-term management  of endoscopically/barium-confirmedGORDN. • Patients who have had dilatation of an oesophageal stricture should remain on long-term full-dose PPI therapy • For all other patients, if symptoms recur following initial treatment, offer a PPI at the lowest dose possible to control symptoms, with a limited number of repeat prescriptions. Discuss using the treatment on an as-required basis to manage symptoms • Refer for consideration of surgery if quality of life remains significantly impaired despite optimal treatment. Surgery of any type is >90% successful although results may deteriorate withtime

Hiatus hernia  Common (30% of over 50s); 50% have GORD. Obesity is a risk factor. The proximal stomach herniates through the diaphragmatic hiatus into the thorax • 80% have a ‘sliding’ hiatus hernia where the gastro-oesophageal junction slides into thechest • 20% have a ‘rolling’ hernia where a bulge of stomach herniates into the chest alongside the oesophagus. The gastro-oesophageal junction remains in the abdomen Management  Treat as forGORD.

Barrett’s oesophagus Usually found incidentally at endoscopy for

symptoms of GORD and caused by chronic GORD. The squamous mucosa of the oesophagus undergoes metaplastic change, and the squamocolumnar junction appears to migrate away from the stomach. The length affected varies. It carries a x40 i risk of adenocarcinoma of the oesophagus, so regular endoscopy is essential. Treatment is with long-term PPIs (e.g omeprazole 20–40mg od) ± laser therapy ± resection. N.R. Barrett (1903–1979)—British surgeon.

Acute gastritis  Mucosal inflammation of the stomach with noulcer. • Type A: affects the entire stomach; associated with pernicious anaemia; pre-malignant • Type B: affects antrum ± duodenum; associated with H.pylori • Type C: due to irritants, e.g. NSAIDs, alcohol, bile reflux Presentation and investigation  Dyspepsia—bp.382 Management • Treat the cause where possible (e.g. vitamin B12 injections; H.pylori eradication; avoidance of alcohol) • Acid suppression—H2 receptor antagonist (e.g. ranitidine, nizatidine) or PPI for4–8wk • Re-endoscope to confirm healing Complications  Haemorrhage, gastric atrophy ± gastric cancer (type Aonly).

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Peptic ulceration Peptic ulceration (PU) is a term which includes both gastric and duo­denal ulceration. Most patients present with dyspepsia (b p.382). Specific ­features of gastric and duodenal ulcers are listed in Table13.5.

Management For patients not takingNSAIDs • Eradicate H.pylori if present (b p.382). Speeds ulcer healing and drelapse; confirm eradication with a urea breath test (duodenal ulcer) or repeat endoscopy (gastric ulcer), and retreat if still present • If H.pylori negative Treat with full-dose PPI (e.g omeprazole 20mg od) for 1–2mo. If gastric ulcer, re-endoscope to check ulcer is healed For patients takingNSAIDs • Stop NSAIDs where possible. If not possible consider changing to a safer alternative (e.g. paracetamol, d dose of NSAID, COX2-selective NSAID) and adding gastric protection with a PPI or misoprostol • Offer full-dose PPI or H2 receptor antagonist (H2RA) therapy for 2mo and, if H.pylori is present, subsequently offer eradication therapy • Check eradication with repeat endoscopy (gastric ulcer) or urea breath test (duodenal ulcer) For all patients • Lifestyle measures Avoid foods (or alcohol) which exacerbate symptoms; eat little and often; avoid eating 5

Risk factors

H.pylori (70–90%) NSAID use (i riskx3–4) Delayed gastric emptying Reflux from the duodenum (iby smoking)

H.pylori (>90%) NSAIDuse Gastric hyperacidity Rapid gastric emptying Smoking Stress (X)

Presentation

May be asymptomatic Epigastric pain worsened by food and helped by antacids or lying flat ± weight loss With complications

May be asymptomatic or spontaneously relapse and remit Epigastric pain typically relieved by food and worse at night ± weight i ± waterbrash (saliva fills themouth) With complications

Examination

In uncomplicated gastric ulceration, examination is usually normal, though there may be epigastric/left upper quadrant tenderness

In uncomplicated duodenal ulceration, examination is usually normal, though there may be epigastric tenderness

Investigation Complications

As for dyspepsia (b p.382) Bleeding: Acute GI bleeding—b p.1076; iron deficiency anaemia—b p.664 Perforated peptic ulcer: DU > GU; GUs may perforate posteriorly into the lesser sac; DUs usually perforate anteriorly into the peritoneal cavity. There may not be a past history of indigestion. Presents with sudden onset severe epigastric pain which rapidly becomes generalized. When a GU perforates into the lesser sac symptoms may remain localized or be confined to the right side of the abdomen. Examination: generalized peritonism with ‘board-like rigidity’. Management: acute surgical admission Pyloric stenosis in adults: duodenal stenosis s to scarring from a chronic DU. Characterized by copious vomiting of food 1–2days old. There may not be a past history of indigestion. Examination: if prolonged vomiting may be evidence of dehydration ± weight d. Succussion splash may be audible. Management: surgical referral for confirmation of diagnosis and surgical relief

Further information NICE Management of dyspepsia in adults in primary care (2004) M www.nice.org.uk

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Gastro-oesophageal malignancy Carcinoma of the oesophagus Common cancer accounting for 7,500 deaths/y in the UK. Most common in patients >60y. Overall, 4:5 85:1. Usually presents late when prognosis is poor. Twotypes: • Squamous cell carcinoma (50%)—predominant form in upper two-thirds of the oesophagus • Adenocarcinoma (50%)—predominant in lower third of the oesophagus. Incidence is increasing. 4:5 85:1 Common risk factors Squamous cell carcinoma • Smoking* • Alcohol • Low fruit/vegetableintake * Risk d to that of a non-smoker 10y after givingup

Adenocarcinoma • Smoking* • Obesity • Low fruit/vegetableintake • GORD—particularly Barrett’s oesophagus (risk i >30x—the longer the affected segment, the higher therisk)

Other risk factors • Previous mediastinal radiotherapy (i x2 for patients treated for breast cancer; i x20 for patients treated for Hodgkin’s lymphoma) • Plummer–Vinson (or Patterson–Kelly) syndrome—oesophageal web and iron deficiency anaemia • Tylosis—rare, inherited disorder with hyperkeratosis of the palms; 40% develop oesophageal cancer Presentation  Short history of rapidly progressive dysphagia affecting solids initially then solids and liquids ± weight loss ± regurgitation of food and fluids (may be bloodstained). Retrosternal pain is a late feature. Other symptoms include hoarseness and/or cough (due to aspiration or fistula formation). Examination may be normal. Look for evidence of recent weight loss, hepatomegaly, and cervical lymphadenopathy. Management  Refer for urgent endoscopy if suspected. Rapid access dysphagia clinics are run in most areas. Specialist management involves resection (treatment of choice but only 1:3 patients are suitable), chemotherapy, radiotherapy, and/or palliation with a stenting tube. Tubes commonly become blocked. Good palliative care is essential—refer early (b p.1028). Overall 8% 5y survival.

Stomach cancer  Stomach cancer causes 75,000 deaths/y in the UK; 95% are adenocarcinomas. Disease affecting older people, with 92% diagnosed >55y; 4 > 5 (5:3). Incidence has more than halved over the past 30y in the UK probably due to improveddiet. Other risk factors  Include: • Geography—common inJapan • Blood groupA • H.pylori infection (not clear if eradication drisk) • Atrophic gastritis

• Pernicious anaemia • Smoking • Adenomatouspolyps • Socialclass • Previous partial gastrectomy

Gastro-oesophageal malignancy

Presentation  Often non-specific. Presents with dyspepsia, weight d, anorexia or early satiety, vomiting, dysphagia, anaemia, and/or GI bleeding. Suspect in any patient >55y with recent onset dyspepsia (within 1y) and/or other risk factors. Examination is usually normal until incurable. Look for epigastric mass, hepatomegaly, jaundice, ascites, enlarged supraclavicular LN (Virchow’s node), acanthosis nigricans. Management  If suspected, refer for urgent endoscopy. In early stages total/partial gastrectomy may be curative. Most present at later stage. Overall 5y survival is15%.

Post-gastrectomy syndromes Abdominal fullness  A feeling of early satiety ± weight loss. Advise to take small, frequentmeals. Bilious vomiting Affects 710% patients post-gastrectomy. Intermittent, sudden attacks of bilious vomiting 15–30min after eating ± epigastric cramping pain relieved by vomiting. Usually settles spontaneously. Metoclopramide or domperidone may be helpful in the interim. If symptoms are severe or fail to settle, request surgical review. Surgical bile diversion or stomach reconstruction may alleviate symptoms. Dumping  Abdominal distension, colic, and vasomotor disturbance (e.g. sweating, fainting) after meals. Affects 1–2% of gastrectomy patients (more common early after surgery—most settle within 6mo). 2types: • Early dumping Due to rapid gastric emptying. Starts immediately after a meal. Consists of: sweating, flushing, tachycardia, palpitations, epigastric fullness, nausea. Occasionally there may be vomiting, diarrhoea ± colicky abdominal pain. Advise: small, dry meals with restricted carbohydrate. Take drinks between meals. If severe, re-refer • Late dumping Due to rapid gastric emptying l hyperglycaemia. The resultant hyperinsulinaemia causes a rebound hypoglycaemia. Starts 1–2h after meals. Consists of:faintness, sweating, tremor, and nausea. Advise patients to d the sugar content of meals, rest for 1h after each meal, and take glucose if symptoms occur. If severe, re-refer Diarrhoea post-gastrectomy 50% of patients who have had a truncal vagotomy or gastrectomy suffer some frequency of defecation; 5% require treatment. The diarrhoea is typically episodic and unpredictable. The exact mechanism is not clear. Treatment is with codeine phosphate or loperamide prn. Antibiotic treatment is occasionally successful—seek expert advice. Surgical measures are rarely necessary. Anaemia  Gastrectomy can result in both vitamin B12 deficiency and iron deficiency anaemia. Prophylactic B12 injections may be advised by the operating surgeon. Many advise iron supplements for life. An annual FBC to monitor for anaemia is advisable. Treat with iron/B12 supplements. Stomach cancer Risk of stomach cancer is i after partial gastrectomy (2xafter 20y, and 7x after45y).

Advice and support for patients Cancer Research UK F 0808 800 4040 M www.cancerhelp.org.uk Macmillan Cancer Support F 0808 808 0000 M www.macmillan.org.uk

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Hernias Irreducible hernia  • Most types of hernia may become irreducible • It may be the first presentation of a hernia or a complication of a longstanding hernia • If obstructed (incarcerated) or strangulated (blood supply to bowel contained within the hernia sac is compromised) the hernia is tender and there are symptoms/signs of small bowel obstruction • If you are unable to reduce a hernia, admit for surgical assessment.

Inguinal hernia Protruberance of peritoneal contents through the abdominal wall where it is weakened by the presence of the inguinal canal. Common condition (4 > 5) which can occur at anyage. Presentation  Lump in the groin ± discomfort on straining/standing for any length of time. There may be a distinct precipitating event (e.g. heavy lifting). Risk factors: chronic cough (e.g. COPD), constipation, urinary obstruction, heavy lifting, ascites, previous abdominal surgery. 2types: • Indirect (80%) Follow the course of the spermatic cord or round ligament down the inguinal canal through the internal inguinal ring (located at the mid-point of the inguinal ligament, 1.5cm above the femoral pulse), and sometimes out through the external inguinal ring into the scrotum/vulva • Direct (20%) Passes through a defect in the abdominal wall into the inguinal canal. Rare in children and more common in the elderly Differential diagnosis of groin lumps  See Table13.6. Examination  Examine the patient standing up. Look for a bulge in the groin above the line of the inguinal ligament. Unless incarcerated the lump should have a cough impulse. Check that you are able to reduce thehernia—sometimes, it is easier if the patient lies down. Ask the patient to reduce the hernia if you cannot. Management  Small hernias often require no treatment. For larger hernias and smaller hernias that are symptomatic, consider referral for surgical repair. Various methods are used—all have a high level of success ( 5. The patient

is usually elderly, although can occur at any age. Peritoneal contents protrude down the femoral canal. Risk of strangulation is high. Presents as a painful lump in the groin and/or small bowel obstruction. Examination  Rounded swelling medially in the groin and lateral to the pubic tubercle; if reducible a soft palpable lump remains after reduction. Management  Always refer for urgent surgical repair. Admit as surgical emergency if obstructed or irreducible.

Hernias

Incisional hernia  Breakdown of the muscle closure in an abdominal wound sometime after surgery. There may be a history of wound sepsis, haematoma, or breakdown. Presents with a bulge at the site of the operation scar ± discomfort. Examination  The hernia is usually visible when the patient stands—it can be made more obvious by asking the patient to cough or straight leg raise whilst lying flat. The margins of the muscular defect are palpable under the skin. Note whether fully reducible ornot. Management  Often reassurance suffices. If obstructed/strangulated or causing discomfort, then refer for surgical assessment. Umbilical hernia Most common in infants (b p.890). In adults para-umbilical hernias, presenting as a bulge adjacent to the umbilicus, may occur due to weakness in the linea alba. 5 > 4. Refer adults for surgical assessment—usually repaired as risk of strangulation is high. Admit as a surgical emergency if obstructed/irreducible. Epigastric hernia  Midline hernia through a defect in the linea alba above the umbilicus. Never contains bowel. Usually symptomless, though occasionally causes epigastric pain ± vomiting. Examination: epigastric mass with cough impulse. Refer for surgical repair. Spigelian hernia  A hernial sac protrudes lateral to the rectus sheath midway between the umbilicus and pubic bone. Presents with discomfort ± vomiting. Refer for surgical repair. Obturator hernia  Hernia protrudes out from the pelvis through the obturator canal. Usually presents with strangulation ± pain referred to the knee. Admit for surgery. Richter hernia  A knuckle of the side wall of the gut gets caught in a hernia sac and becomes strangulated but the bowel is not obstructed. Presents with abdominal pain which rapidly becomes worse ± shock. Admit as for acute abdomen; diagnosis is usually made at surgery. Table13.6  Differential diagnosis of groinlumps Position relative to the skin In the skin Deep to the skin

Position relative to the inguinal ligament Groin lump

Above

Below

Lipoma, fibroma, haemangioma, andotherskin lumps

     

Femoral or inguinal lymphnodes

Saphena varix of the femoral vein

Femoral artery aneurysm

Femoral hernia

Inguinal hernia

0 The inguinal ligament runs from the pubic tubercle medially to the anterior superior iliac spine laterally.

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Appendicitis and small boweldisease Acute appendicitis Most common surgical emergency in the UK.

Peak age: 10–30y. Presents with central abdominal colic that progresses to localize in the right iliac fossa. Pain is worse on movement (especially coughing, laughing) and associated with anorexia, nausea ± vomiting, dysuria, constipation or rarely diarrhoea. Assessment  Watch for discomfort on walking (walk stooped). May be flushed and unwell—pyrexial (737.5°C); furred tongue and/or foetor oris; tenderness, rebound tenderness and guarding in the right iliac fossa (especially over McBurney’s point—two-thirds of the distance between the umbilicus and anterior superior iliac spine); pain in the right iliac fossa on palpation of the left iliac fossa (Rovsing’s sign). Urinalysis is normal or +ve for protein and/or leucocyte esterase but–ve for nitrites. Differential diagnosis • Urological cause, e.g. UTI, testicular torsion • Mesenteric adenitis • Gynaecological cause (e.g. pelvic • Gastroenteritis inflammatory disease; ectopic pregnancy) • Meckel’s diverticulum • Non-abdominal cause, e.g. otitis media, • Intussusception diabetic ketoacidosis, pneumonia • Crohn’s disease Management  Admit as a surgical emergency—expect to be wrong 750% the time. Complications: generalized peritonitis s to perforation; appendix abscess; appendix mass; subphrenic abscess; female infertility. Subphrenic abscess  Rarely follows 7–21d after generalized peritonitis— particularly after acute appendicitis. Presents with general malaise, swinging fever, nausea, and weight d ± pain in the upper abdomen radiating to the shoulder tip. Breathlessness can be associated due to reactive pleural effusion or lower lobe collapse. Examination: subcostal tenderness ± liver enlargement. FBC—i WCC. If suspected admit for surgical assessment.

• Appendicitis in pregnancy  Appendicitis affects 1:1,000 pregnan-

cies. Mortality is i and perforation more common (15–20%). Fetal mortality is 5–10% for simple appendicitis; 30% when there is perforation. Due to the pregnancy, the appendix is displaced—pain is often felt in the paraumbilical region or subcostally. Admit immediately if suspected. Children with appendicitis  Symptoms/signs of appendicitis may be atypical—especially in very young children— as children localize pain poorly and signs of peritonitis can be difficult to elicit. • If unsure of diagnosis and the child is unwell,admit • If unsure of diagnosis and the child is well, either arrange to review a few hours later, or ask the carer to contact you if there is any deterioration, or change in symptoms Mesenteric adentitis  Inflammation of the mesenteric LNs, causing abdominal pain in children. May follow URTI. Can mimic appendicitis. Check MSU to exclude UTI. If guarding/rebound tenderness, refer for acute surgical assessment. Settles spontaneously with simple analgesia and fluids. If not settling in 1–2wk refer for paediatric assessment.

Appendicitis and small boweldisease

Meckel’s diverticulum Remnant of the attachment of the small

bowel to the embryological yolk sac. It is 2inches (75cm) long, 72ft (60cm) proximal to the appendix and present in 2% of the population. AMeckel’s diverticulum may not cause any problems or cause an appendicitis-like picture; acute intestinal obstruction, or GI bleeding. Symptoms can occur at any age but are most common in children. J.F. Meckel (1781–1833)—German anatomist.

Intussusception  bp.891 Coeliac disease  bp.412 Crohn’s disease  bp.414 Obstruction and ischaemia  bp.400 Adhesions  Arise as a result of intra-abdominal inflammation. Bowel loops become adherent to each other, omentum, mesentery, and the abdominal wall. Fibrous bands may form connecting adjacent structures. Presents with abdominal pain ± obstruction. Causes: surgery; intra-abdominal sepsis (e.g. appendicitis, cholecystitis; salpingitis); inflammatory bowel disease; endometriosis. Refer to a surgeon. Treatment is difficult, as any surgery may result in new adhesions; conservative management with analgesia and stool softeners is preferred. Laparoscopic, or rarely open division of adhesions is occasionally necessary. Intestinal non-Hodgkin’s lymphoma  The majority of intestinal NHLs

are B-cell type lymphomas, but coeliac disease is associated with T-cell intestinal lymphoma. Abdominal symptoms: non-specific abdominal pain (70–80%); perforation (up to 25%); bowel obstruction; abdominal mass; intussusception; malabsorption (usually lymphoma associated with coeliac disease), or alteration in bowel habit (small intestine NHL may present like Crohn’s disease). Systemic symptoms: weight d (30%), fatigue, sweats, unexplained fevers. 0Lymphadenopathy and hepatosplenomegaly are usually absent. Management  (b p.680) Gastric lymphoma may remit with treatment of H.pylori infection.

Carcinoid tumours  Slow-growing tumours of low malignancy, which arise from neuroendocrine cells or their precursors. Incidence: 3–4/100,000. Peak age: 61y. 5 > 4. 60% are in the midgut (especially appendix and terminal ileum). Examination may reveal an abdominal mass and/or enlarged liver. Rarely presents with bowel obstruction. Ileal carcinoids are multiple in 30%. Non-intestinal sites: lung, testes, andovary. Carcinoid syndrome Affects 90% long-term survival. Without screening, most tumours are detected at advanced stages and overall 5y survival is 850%. Screening aims to detect colorectal cancer at an early stage to i survival chances. Screening test  Faecal occult blood (FOB) test kits are sent every 2y to all patients aged 60–74y with instructions for completion/return. The test kit has 3 flaps, each with 2 windows underneath. 2 samples are taken from a bowel motion and spread onto the 2 windows under the first flap using the cardboard sticks provided. The flap is then sealed and the process repeated using the remaining 2 flaps for the subsequent 2 bowel motions. Once all 6 windows have been used, the kit is returned. Kits must be returned 4wk. Patients’ perceptions of diarrhoea vary widely. Clarify what is meant. Chronic diarrhoea affects 74–5% of adults in the UK. There are many causes (see Table13.11), and all patients require investigation. Careful history is vital. Symptoms suggestive of organic disease • History of 1) and other factors that might be contributing to it. Askabout: • Onset and nature of symptoms 0 Always consider faecal incontinence when patients present with anal soreness and/or itching • Bowel habit including timing and frequency of incontinence • Difficulties with toileting and help available • Other medical conditions • Diet • Medication • Social circ*mstances • Persistent change in bowel habit to looser stools may be a sign of GI malignancy—bp.399.

Examination  General and rectal examination (to detect abnormalities of anal tone, local anal pathology, e.g. rectal prolapse, and constipation causing overflow incontinence). Further examination depends on age group and history, e.g. cognitive assessment if suspected cognitive deficit; neurological examination if d analtone. Primary care management Treatment ofcause • Clear any constipation/faecal loading (b p.378)—use rectal preparations initially to clear faecal load. If unsuccessful/rectal preparations are inappropriate, then switch to oral laxatives. Take steps to prevent recurrence, e.g. add fibre to diet, i fluid intake, consider regular laxatives • Treat other reversible causes, e.g. infective diarrhoea,UC • Consider alternatives to any contributing medications, e.g. tranquillizers General measures where cause cannot be treated • Advise fluid intake of at least1.5L/d • Encourage bowel emptying after a meal—advise patients to assume a seated/squatting position and not tostrain • Ensure that toilet facilities are private, accessible, and safe—refer for OT assessment ifneeded

Faecal incontinence

• Manipulate diet to promote optimal stool consistency and predictable bowel emptying. Afood/fluid diary may be helpful. Only change one food at a time. Consider referral to a dietician • If stool must be in the rectum at a set time (e.g. when a carer is there), manipulate bowel action with pr/po laxatives and/or loperamide • If loose stools, consider treatment with loperamide, co-phenotrope, or codeine phosphate, prn or continuously. When using loperamide, introduce at a very low dose (consider syrup for doses 1 meal/d containing gluten for≥6wk • Other tests Also consider FBC, ESR/CRP, B12, folate, ferritin, LFTs, Ca2+, TFTs, and stool sample for M,C&S (if diarrhoea) • Selective IgA deficiency is more common amongst people with coeliac disease (2.6%) than the general population (0.4%). People who are IgA-deficient have false–ve results with IgA TTG/EMA testing. When there is strong clinical suspicion and coeliac serology is negative, check serum IgA levels; if deficient, request IgG TTG/EMA antibody testing.

Initial management  Refer for specialist reviewif: • +ve serology—duodenal biopsy showing villous atrophy is diagnostic • Strong clinical suspicion of coeliac disease but–ve serology • Unwilling to reintroduce gluten to diet to enable serological testing Gluten-free diet  Is the cornerstone of the management of coeliac disease and should be followed life-long. Patients should avoid proteins derived from wheat, rye, or barley. X Avoidance of oats is controversial. Refer to a dietician for specialist advice. Coeliac UK provides a directory of approved products as well as recipes for those on gluten-freediets. Prescriptions for gluten-free foods  Prescribe adequate gluten-free foods (see Table13.14), marking prescriptions ‘ACBS’. Add deficient nutrients, e.g. iron, folic acid, calcium until established on a gluten-freediet. Failure to respond to diet The most common reason is continued ingestion of gluten (intentional or inadvertent). Re-refer to dieticians. If symptoms recur after a period of remission, re-refer for specialist review.

Pneumococcal

vaccination  Pneumococcal common 2o to hyposplensim—advise vaccination.

infection

is

more

Follow-up  Every 6–12mo in a specialist clinic or by a GP under shared

care arrangements. Routine checks include:symptoms, weight, and blood tests (Hb, B12, folate, iron, albumin, Ca2+, TTG or EMA antibodies).

Long-term complications  Are almost eliminated by strictdiet: • Osteoporosis—consider DEXA scan at diagnosis, after 3y on a gluten-free diet (if abnormal baseline DEXA), at menopause for 5, aged 55y for 4, or if fragility fractureG • Malignancy—lymphoma or carcinoma of the small intestine. Rare—if suspected, refer urgently for specialist review

Coeliac disease

Table13.13  Presentation of coeliac disease Symptoms and signs Chronic/intermittent diarrhoea(50%) Failure to thrive/faltering growth in children Recurrent abdominal pain/ cramping/bloating Other persistent unexplained GI symptoms, e.g. nausea/ vomiting Sudden or unexpected weightd Unexplained anaemia (irondeficiency orother) Genetic predisposition First-degree relative (parent,sibling,child) Down’s/Turner syndrome

Associated conditions GI Dental enamel defects Mouthulcers Irritable bowel syndrome Microscopic colitis Persistent/unexplained constipation Unexplained, persistent i in liver enzymes (usually normalize in 37.5oC or tachycardia >90bpm, admit as an acute emergency. If persistent, unexplained diarrhoea lasting >4wk and/or persistent abdominal pain, refer for urgent further investigation to exclude GI malignancy and establish diagnosis. Table13.15  Assessing the severity of ulcerative colitis Severity

Symptoms

Mild

6 liquid stools/d Severe rectal bleeding Any systemic disturbance (i pulse rate >90bpm, pyrexia >37.5oC, i ESR, i WCC, d Hb Crohn’s) Miscellaneous—thromboembolism, osteoporosis (Crohn’s); amyloidosis (Crohn’s)

Examination

Abdominal + rectal examination—abdominal tenderness. Anal and perianal lesions (pendulous skin tags, abscesses, fistulae) and/or mass in the right iliac fossa are characteristic of Crohn’s disease General examination—clubbing, aphthous ulcers in the mouth (Crohn’s), signs of weight loss, anaemia, or hypoproteinaemia

Investigation

Blood: FBC (anaemia, i WCC), ESR (i when disease is active), eGFR, LFTs (including serum albumin). In severe UC, CRP >45g/dL after 3d steroid treatment indicates high (785%) risk for colectomy. Stool: M,C&S (including C.difficile) to exclude infection AXR: consider to clarify extent of disease, exclude toxic megacolon (transverse colon diameter >5cm) or bowel obstruction, and/or identify proximal constipation Proctoscopy: inflammation and shallow ulceration extending proximally from the anal margin suggests UC

UC and Crohn’s disease are rare in childhood. Presentation is variable and can be with non-specific features (e.g. failure to thrive), GI symptoms (e.g. malabsorption, bloody diarrhoea, acute abdomen) or complications (e.g. arthropathy or iritis). If suspected refer for confirmation of diagnosis and specialist management.

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Management of ulcerative colitis  See BNF1.5. Assessing severity  See Table13.15, b p.414. Active disease • Mesalazine 2–4g daily. Topical 5-ASA derivatives are a useful adjunct if troublesome rectal symptoms • Add steroids (prednisolone 40mg od po + rectal preparation) if prompt response is needed or mesalazine is unsuccessful. Review frequently and d dose over 8wk. Rapid withdrawal i risk of relapse • Azathioprine is added if the patient is having recurring attacks despite mesalazine maintenance frequent steroids (≥2 courses/y), disease relapses as dose of steroid is d, or relapse 8x/d) ± bleeding • Dramatic weightloss • Fever >37.5°C, tachycardia >90bpm, or other signs of systemic disease Maintenance treatment Follow-up in s care is routine. Most patients require life-long therapy. Mainstays of treatment are 5-ASA derivatives (e.g. mesalazine 1–2g/d or balsalazide 3g/d). Use a rectal formulation (e.g mesalazine 1g/d PR) if disease is confined to the rectum or descending colon. Long-term treatment d risk of colonic cancer by 75%. 10% are intolerant to 5-ASA derivatives—alternative is azathioprine (consultant supervision required). Treat proximal constipation with stool-bulking agents or laxatives. NSAIDs can precipitate relapse soavoid. Surgery  Last resort—but should not be delayed if severe colitis and failing to respond to medical therapy. 20–30% of patients with pancolitis require colectomy—1:3 develop pouchitis (non-specific inflammation of the ileal reservoir) within 5y of surgery. Prognosis  At any time 50% are asymptomatic, 30% have mild symptoms, and 20% moderate/severe symptoms. 3mo should always be avoided • Elemental or polymeric diets for 4–6wk can be a useful adjunct or alternative to steroid treatment—take consultant advice

Inflammatory bowel disease

• Other treatments (consultant supervision) include metronidazole, azathioprine, anti-tumour necrosis factor (infliximab or adalimumab) • Surgery is an option if medical treatment has failed. 50% need surgery 8x/d) ± bleeding • Dramatic weightloss • Bowel obstruction • Fever/other signs of systemic disease 0 For disease elsewhere, take specialist advice. Maintenance treatment Follow-up in s care is routine. Treatment is aimed at d impact of the disease. The most effective measure is to stop smoking. Mesalazine has limited benefit. It is ineffective at doses 8y, onset in childhood/adolescence, age >45y, FH of colon cancer, extensive colitis, sclerosing cholangitis. Prevention: screening with colonoscopy Frequency depends on severity of the disease and duration of symptoms Sclerosing cholangitis Fibrosis and stricture of intra- and extrahepatic bile ducts. Presents with obstructive jaundice

Crohn’s Intra-abdominal abscess Intestinal stricture Common—may require surgery Toxic megacolon Rare (seeUC) Bowel obstruction Fistula formation Perianal disease Malignancy Large and small bowel cancer—5% 10y after diagnosis Osteoporosis

Psychological effects Chronic, life-long conditions which have major impact on work and domestic life. Self-help groups can be useful

Further information NICE Crohn’s disease (2012) M www.nice.org.uk British Society of Gastroenterology Guidelines for the management of inflammatory bowel disease in adults (2004) M www.bsg.org.uk

Advice and support for patients  Crohn’s and Colitis UK F 0845 130 2233 (info); 0845 130 3344 (support) M www.crohnsandcolitis.org.uk

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Irritable bowel syndrome Irritable bowel syndrome (IBS) is a chronic (>6mo) relapsing and remitting condition of unknown cause, with symptoms including:Abdominal pain or discomfort; Bloating; and Change in bowelhabit. It is a diagnosed on symptoms with no confirmatory test and no cure. Extremely common. Lifetime prevalence ≥20%, although 750% never consult a GP. 5 > 4 (2.5:1). Symptoms can appear at anyage. Diagnosis of IBS  Abdominal pain or discomfort thatis: • Relieved by defecation,or • Associated with altered bowel frequency or stoolform and ≥2 of the following: • Altered stool passage (straining, urgency, incomplete evacuation) • Abdominal bloating (5 > 4), distension, tension, or hardness • Symptoms made worse byeating • Passage ofmucus Other commonly associated symptoms include  Lethargy, nausea, backache, and bladder symptoms.

Differential diagnosis • Pelvic inflammatory disease • Colonic carcinoma • Endometriosis • Coeliac disease • GI infection • Inflammatory bowel disease • Thyrotoxicosis (Crohn’s diseaseorUC) Investigation  A diagnosis of exclusion. How far to investigate is a clinical judgement weighing risks of investigation against possibility of serious disease. Judgement is based on age of the patient, family history, length of history, and symptom cluster. • Patients 40y Colonic cancer must be excluded for any patient with a persistent, unexplained change in bowel habit—particularly towards looser stools (b p.399) • Other investigations to consider • Thyroid function tests if other symptoms/signs of thyroid disease • Stool samples to exclude GI infection if diarrhoea • Endocervical swabs for chlamydia • Colonoscopy to exclude inflammatory bowel disease • Laparoscopy to exclude endometriosis Referral  To gastroenterology/colorectal surgeryif: • Passing blood (except if from an anal fissure or haemorrhoids)—U • Abdominal, rectal, or pelvicmass—U • Unintentional/unexplained weight loss—U/S • Positive inflammatory markers and/or anaemia—U/S • >40y with new symptoms—U (if age >60y)/S/R • Change in symptoms, especially if >40y—U (if age >60y)/S/R • Atypical features (i.e. not those listed above)—U/S/R • Family history of bowel or ovarian cancer—R • Patient is unhappy to accept a diagnosis of IBS despite explanation—R U = urgent; S = soon; R = routine.

Irritable bowel syndrome

Treatment  Reassure. Information leaflets are helpful. Encourage

lifestyle measures, stress d, leisure time, and regular physical exercise. Diet  Encourage patients to have regular meals and take time to eat. Avoid missing meals or leaving long gaps between eating. • Drink ≥8 cups of fluid/d, especially water. Restrict tea/coffee to 3cups/d. d intake of alcohol and fizzy drinks • d intake of high-fibre foods (e.g. wholemeal/high-fibre flour and breads, cereals high in bran, and whole grains such as brownrice) • d intake of ‘resistant starch’ found in processed or re-cookedfoods • Limit fresh fruit to 3x 80g portions/d • For diarrhoea, avoid sorbitol, an artificial sweetener • For wind and bloating consider increasing intake of oats (e.g. oat-based breakfast cereal or porridge) and linseeds (≤1 tablespoon/d) • Up to 50% may be helped by exclusion of certain foods (especially patients with diarrhoea—predominant disease). Diaries may help identify foods that provoke symptoms. Common candidates are dairy products, citrus fruits, caffeine, alcohol, tomatoes, gluten, and eggs. Refer to dietician for exclusiondiet Specific measures • Probiotics Some evidence of effectiveness. Try a 4wktrial • Fibre/bulking agents Constipation-predominant IBS. Bran can make some patients worse. Ispaghula husk is better tolerated. Laxatives are an alternative but avoid use of lactulose • Antispasmodics (e.g. mebeverine, peppermint oil) All equally effective. If no response in a few days, switch to another—different agents suit different individuals. Once symptoms are controlled useprn • Antidiarrhoeal preparations (e.g. loperamide) Avoid codeine phosphate as may cause dependence. Use prn for patients with diarrhoea-predominant disease. Use pre-emptive doses to cover difficult situations (e.g. air travel) • Antidepressants There is evidence that low-dose amitriptyline, e.g. 10mg nocte, is effective. SSRIs are less effective unless the patient is overtly depressed. Withdraw if no response after4–6wk • Psychotherapy and hypnosis Some effect in trials. Reserve for cases that have failed to respond to more conventional treatment Failure to respond to treatment  Consider another diagnosis—review history and examination ± refer for further investigation.

Prognosis  >50% still have symptoms after5y. Further information NICE Irritable bowel syndrome in adults:diagnosis and management of irritable bowel syndrome in primary care (2008) M www.nice.org.uk

Advice and support for patients The IBS Network F 0872 300 4537 M http://theibsnetwork.org

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Jaundice and abnormal liver function Jaundice  Yellow pigmentation of the tissues due to excessive bile pigment. Clinical jaundice appears when serum bilirubin >35micromol/L. Causes • i production of bilirubin (pre-hepatic) Haemolytic anaemia, drug-induced haemolysis, malaria, Gilbert’s/Crigler–Najjar syndrome • Defective processing (hepatic) Hepatitis, cirrhosis • Blocked excretion (obstructive) Gallstones, pancreatic cancer, primary biliary cirrhosis, primary sclerosing cholangitis, cholangiocarcinoma, sepsis, enlarged porta hepatis (e.g. s to lymphoma) History  Patients presenting with jaundice may have no other symptoms. General symptoms include tiredness, nausea, and pruritus. Ask about colour of the stools and urine (dark urine suggests conjugated hyperbilirubinaemia and hepatobiliary disease). Check alcohol consumption. Examination  Mild jaundice is best seen by examining the sclerae in natural light. Look for signs of chronic liver disease and examine the abdomen for masses and hepatomegaly. Investigations  Initially check FBC and liver function tests—see Table13.18. Further investigations depend on the results. Management  Treat the cause of the jaundice. Most patients (except those with Gilbert’s syndrome or self-limiting viral hepatitis) will require specialist referral. Refer patients with pre-hepatic and hepatic jaundice to a hepatologist or gastroenterologist; refer patients with post-hepatic (obstructive or cholestatic) jaundice to a general or hepatobiliary surgeon.

Neonatal jaundice  bp.868 Abnormal liver function Raised AST/ALT/GGT in isolation Liver enzymes can i transiently as a result of viral infection, drugs, or alcohol. ALT tends to be more raised in viral and autoimmune hepatitis; AST tends to be more raised in patients with fatty liver; raised GGT is particularly associated with alcohol excess. • Check medication including herbal medicines • Stop alcohol • RepeatLFTs: • In 1mo if AST/ALT are 70%; total iron binding capacity d. Refer. Liver biopsy is diagnostic. Venesection returns life expectancy to normal. Secondary haemochromatosis Iron overload from frequent transfusions, e.g. for haemolysis. Specialist management with chelation therapy to i iron excretion is required. α1- antitrypsin deficiency  bp.425 Wilson’s disease (hepatolenticular degeneration) Rare, autosomal recessive disorder. Defective biliary copper excretion l accumulation of copper in the liver, brain, kidney, and cornea. Treatment is with penicillamine. Liver transplantation is the only treatment if presentation is with acute liver failure. S.A.K. Wilson (1878–1937)—British neurologist.

Information and support for patients British Liver Trust F 0800 652 7330 M www.britishlivertrust.org.uk Primary Biliary Cirrhosis Organization M www.pbcers.org Wilson’s Disease Association M www.wilsonsdisease.org

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Liver failure and portal hypertension ‘Jaundice is the disease that your friends diagnose’ Aphorisms, Sir William Osler (1849–1920)

Cirrhosis  The liver is replaced by fibrotic tissue and regenerating nod-

ules of hepatocytes.

Causes  • Unknown(30%) • Alcohol(25%) • Viral hepatitis • Primary biliary cirrhosis • Haemochromatosis

• Wilson’s disease • Budd–Chiari syndrome • Chronic active hepatitis • α1-antitrypsin deficiency

Presentation  Variable. May be an incidental finding. Symptoms/signs: • Gynaecomastia • Hepatomegaly (although liver • Testicular atrophy becomes small and hard in late • Clubbing stages) • Xanthelasma/xanthomata • Spidernaevi • Portal hypertension • Dupuytren’s contracture • Splenomegaly • Palmar erythema

Acute liver failure  Presents with sudden onset of severe illness. • Jaundice • Hypoglycaemia • Hepatic encephalopathy—ranges from mild confusion and irritability, through drowsiness and increasing confusion, tocoma • Haemorrhage—due to deranged clotting factors • Ascites—hepatosplenomegaly and ascites are not usually prominent • Infection • Nausea ± vomiting • iBP • Foetor hepaticus (sweet smell on the breath) Causes In previously healthy patients: tetrachloride) • Viral hepatitis • Heatstroke • Weil’s disease • Paracetamol overdose • Budd–Chiari syndrome • Halothane • Pregnancy • Idiosyncratic drug reactions • Wilson’s disease • Fungal/planttoxins • Reye’s syndrome • Malignant infiltration • Chemical exposure (e.g. carbon In patients with chronic liver disease: • Diuretics and/or electrolyte imbalance • Infection • Alcoholbinges • GI bleeding • Constipation • Sedation Management  Admit as emergency to a hepatologist/gastroenterologist unless an expected terminal event. Prognosis is poor (50% present as a result of investigation for abnormal LFTs (b p.420). Characteristic ‘bright’ appearance on liverUSS • Less frequently presents with a smoothly enlarged liver or symptoms— nausea, vomiting, abdominal pain, fat embolus (maybefatal) Broadly divides into 2forms: Alcohol associated fatty liver disease • Defined as fatty liver disease with daily ethanol consumption >20g (5) or 30g(4) • Typically serum AST and ALT are i, but serum AST > serumALT • GGT may be i, and alk. phos may also be i but usually serumAST • GGT may be i and ALP may also be i but usually 500ng/mL in a patient with known cirrhosis is almost certainly diagnostic. The most important prognostic factors are the number and size of the liver lesions and the presence of vascular involvement. 95% of patients with cirrhosis have disease too extensive for curative surgery, or their severely compromised liver function makes radical surgery inappropriate. 50% of patients without cirrhosis have resectable tumours. Surgery may be combined with liver transplantation. Inoperable tumours may be treated with hepatic artery ligation or embolization. Tumours respond poorly to chemo- or radiotherapy. Overall prognosis  Patients with cirrhosis—median survival 3mo; patients without cirrhosis—median survival1y. Cholangiocarcinoma  Rare adenocarcinoma of the biliary tract. May be associated with UC. Typically presents in patients >60y with jaundice, RUQ pain and weight loss. The only effective treatment is surgery, which is only possible in 710–20% of patients. Selected fit patients with unresectable disease may be offered palliative chemotherapy or enrolment in a clinical trial. Median survival4–6mo. Secondary tumours  The most common type of liver tumours—usually signalling late disease. Presentation: hard, enlarged, knobbly liver ± RUQ pain ± jaundice (late). If found and no history of malignancy refer to oncology/ general surgery for urgent referral to find the primary. 1° tumours commonly metastasizing to the liver Lung, breast, large bowel, stomach, uterus, pancreas, carcinoid, lymphoma, leukaemia.

Advice and support for patients Cancer Research UK F 0808 800 4040 M www.cancerhelp.org.uk Macmillan Cancer Support F 0808 808 0000 M www.macmillan.org.uk British Liver Trust F 0800 652 7330 M www.britishlivertrust.org.uk

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Gallbladder disease Gallstones  Gallstones are increasingly common. 9% of 60y olds have them and prevalence i withage. Other risk factors • Gender (5>4) • Body weight—prevalence i with weight; also associated with rapid weightd • Race—in the USA, Native American > Hispanic > white >black • Affluency • Pregnancy (and possibly HRT but not COCpill) • Alcohol is protective • Diet—vegetarian diet is protective Associated conditions • Haemolysis • DM • Hypertriglyceridaemia

• Cirrhosis • Crohn’s disease • Partial gastrectomy

Drugs which cause gallstones  Clofibrate (and other fibric acid derivatives); octreotide (somatostatin analogue). Presentation  Gallstones are blamed for many digestive symptoms—they are probably innocent in most cases. 70% of stones in the gall bladder do not cause symptoms. Common presentations—see Table13.19. Management of gallstones • Advise the patient to stick to a low-fatdiet • Refer for surgical review ± further evaluation (e.g. ERCP—endoscopic retrograde cholangiopancreatography) • Gallstones can be removed by cholecystectomy (laparoscopic or open) or ERCP or may be dissolved with ursodeoxycholic acid (stones 40y with right upper quadrant pain, anorexia, weight d, and jaundice. Surgical resection offers the only hope of cure but disease is usually advanced at presentation. Selected fit patients with unresectable disease may be offered palliative chemotherapy or enrolment in a clinical trial. Prognosis ispoor.

Information and support for patients British Liver Trust F 0800 652 7330 M www.britishlivertrust.org.uk

Gallbladder disease

Table13.19  Presentation and management of gallstone disease Presentation

Management

Clear-cut attacks of severe upper abdominal pain which may radiate l back/shoulder tip, lasting ≥30min and causing restlessness ± jaundice ± nausea or vomiting Examination: tenderness ± guarding in the right upper quadrant (i on deep inspiration— Murphy’s sign)

Treat acute attacks with pethidine (50mg IM/po) or naproxen (500mg po) + prochlorperazine 12.5mg IM or domperidone 10mg po/PR for nausea Admit if: uncertain of diagnosis, inadequate social support, persistent symptoms despite analgesia, suspicion of complications, and/or concomitant medical problems (e.g. dehydration, pregnant, DM, Addison’s) Investigate: for gallstones with abdominal USS to prove diagnosis when the episode has settled Differential diagnosis: any cause of acute abdomen Treat: gallstones to prevent recurrence

Pain and tenderness Acute cholecystitis/ in the right upper quadrant/epigastrium ± cholangitis vomiting Examination: tenderness ± guarding in the right upper quadrant ± fever ± jaundice

Treatment: broad-spectrum antibiotic (e.g. ciprofloxacin) and analgesia as for biliary colic Admit if: generalized peritonism, diagnosis uncertain, very toxic, concomitant medical problems (e.g. dehydration, DM, Addison’s, pregnancy), inadequate social support, or not responding to medication Empyema occurs when the obstructed gall bladder fills with pus. Presents with persistent swinging fever and pain. Usually requires cholecystectomy ± surgical drainage Investigate and follow up to prevent recurrence as for biliary colic

Pancreatitis

b p.430

b p.430

Gallstone ileus

Occurs usually after an attack of cholecystitis. Astone perforates from the gall bladder into the duodenum and impacts in the terminal ileum causing bowel obstruction

b p.400

Chronic cholecystitis

Vague intermittent abdominal discomfort, nausea, flatulence, and intolerance of fats

Investigate for gallstones with abdominal USS to prove the diagnosis

Biliary colic

Differential diagnosis: reflux, IBS, upper GI tumour,PU Refer for treatment of gallstones

Jaundice

Refer for same day or urgent specialist Obstructive surgical assessment (depending on clinical jaundice—b p.420 ± right upper quadrant pain state)

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Pancreatitis Acute pancreatitis Premature activation of pancreatic enzymes

results in autodigestion and tissue damage. Most episodes are mild and self-limiting but 1:5 patients have a severe attack. Overall mortality 75–10%. May be recurrent. Causes  In 10% patients no cause is identified. • Common causes (80%): gallstones, alcohol • Rarer causes: • Hyperlipidaemia • Drugs (e.g. azathioprine) • Pancreas divisum (normal variant • Trauma in 7–8% of the white population) • Pancreatic tumours • Familial pancreatitis • Post-ERCP • Vasculitis • Viral infection (mumps, HIV, • Ischaemia or embolism CoxsackieB) • Pregnancy • Mycoplasma infection • End-stage renal failure • Hypercalcaemia Presentation • Poorly localized, continuous, boring epigastric pain which i over 71h— often worse lying down ± radiation to the back(50%) • Nausea ± vomiting Examination • General Tachycardia, fever, shock, jaundice • Abdominal Localized epigastric tenderness or generalized abdominal tenderness; abdominal distension ±d bowel sounds; evidence of retroperitoneal haemorrhage (periumbilical and flank bruising—rare) Management  Admit as an acute surgical emergency. Prior to transfer, give analgesia with pethidine (morphine may induce spasm of the sphincter of Oddi). Complications  Delayed complications may present in general practice— suspect if persistent pain or failure to regain weight or appetite. Complications include: • Pancreatic necrosis • Pseudocyst—localized collection of pancreatic secretions • Fistula/abscess formation • Bleeding or thrombosis Prevention of further attacks • Avoid factors that may have caused pancreatitis, e.g. alcohol,drugs • Advise patients to follow a low-fatdiet • Treat reversible causes, e.g. hyperlipidaemia, gallstones

Chronic pancreatitis  Chronic inflammation of the pancreas results in gradual destruction and fibrosis of the gland ± loss of pancreatic function l malabsorption andDM. Cause  Alcohol is responsible for most cases. More rarely: familial; CF; haemochromatosis; pancreatic duct obstruction (gallstones/pancreatic cancer); hyperparathyroidism.

Pancreatitis

Presentation • Constant or episodic epigastric pain, radiating to the back and relieved by sitting forwards • Weightd • Vomiting • DM • Weakness • Chronic poorhealth • Jaundice • Steatorrhoea Management  Refer to gastroenterology. Treatment: • Diet Low-fat, high-protein, high-calorie diet with fat-soluble vitamin supplements. Refer to dietician • Pancreatic enzyme supplementation (e.g. Creon® capsules pre-meals) May improve diarrhoea • Alcohol abstinence • Pain control Provide analgesia—beware of opioid abuse. Consider referral for coeliac plexusblock • Surgery Pancreatectomy or pancreaticojejunostomy for pancreatic duct stricture, obstructive jaundice, unremitting pain, or weightloss • Diabetes management

Pancreatic insufficiency  Global d function of the pancreas. Causes: • Child Cystic fibrosis • Adult Chronic pancreatitis, pancreatic tumour, pancreatectomy, total gastrectomy Presentation  Malabsorption (frequent loose, odorous stools ± abdominal pain), weight loss or failure to thrive,DM. Management  Take specialist advice. Treat the underlying cause. Treat associated DM. Suppplement digestive enzymes (e.g. with Creon®).

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Pancreatic tumours Pancreatic cancer accounts for 3% of all malignancies, causing 7,800 deaths/y in the UK. 80% of cases occur in patients >60y. 4 > 5(3:2).

Risk factors • Smoking—causes 25–30% of pancreatic cancers in the UK. Risk returns to non-smoker levels 10–20y after cessation • Chronic pancreatitis—usually related to excess alcohol • Type II (non-insulin-dependent) DM—relative risk81.8 • Obesity—i riskby19% • Genetic—5% pancreatic cancers are hereditary; characterized by presentation aged 90% are benign. 7–8% are associated with MEN Isyndrome. Presents with episodes of hypoglycaemia, especially when exercising or fasting. i appetite and frequent food intake to avoid hypoglycaemia often results in substantial weightgain. Somatostatinoma  Uncommon islet cell tumour. Most are large tumours (>5cm) in the head/body of the pancreas. Presents with gallstones, steatorrhoea, andDM. 0 Extrapancreatic somatostatinomas can present in association with neuro­ fibromatosis type Iand phaeochromocytoma. Verner–Morrison syndrome An intestinal vasointestinal peptide (VIP)producing tumour results in profuse watery diarrhoea l dehydration, metabolic acidosis, and d K+. Also associated with insulin resistance and impaired glucose tolerance. VIPomas account for 90mL/min/1.73m2 • eGFR 60–90mL/min/1.73m2 does not indicate CKD unless additional markers of damage are present—persistent microalbuminuria, persistent proteinuria, persistent microscopic haematuria, or structural abnormality, e.g. polycystic kidneys • eGFR 40y). Many elderly patients have a GFR 5mmol/L). Causes: See Table14.2. Treat thecause.

• Plasma potassium >6.5mmol/L needs urgent treatment. • Check it is not an artifact, e.g. due to haemolysis inside thebottle • Admit for investigation of cause and treatment ECG changes associated with hyperkalaemia  Tall tented T-waves; small P-wave; wide QRS complex becoming sinusoidalVF.

Hypokalaemia Low serum potassium (2.5mmol/L and no symptoms, give oral potassium supplement. 0 If the patient is taking a thiazide diuretic, hypokalaemia >3.0mmol/L rarely needs treating. • Plasma potassium 150micromol/L)

d creatinine i urea (6.7mmol/L)

Renal disease/renal failure Drugs (e.g. trimethoprim, probenecid, cimetidine, potassiumsparing diuretics) Large muscle bulk Muscle breakdown (e.g. muscular dystrophy)

Muscular dystrophy (late stage) Myasthenia gravis

Renal failure GI bleeding High-protein diet Drugs—highdose steroids, tetracycline Dehydration

d urea (5mmol/L)

d potassium (145mmol/L) (200mg/mmol; microscopy for red cells, casts; Blood—U&E, creatinine, eGFR, albumin (15% between tests or >5mL/ min/1.73m2 in 10mL/min/1.73m2 in 100mg/mmol or ACR >70mg/mmol)

Renal failure

• Sudden d in eGFR (>15%) and UTI excluded • Persistent microscopic haematuria and 50y) • Functional consequences of CKD, i.e. anaemia (140/90 on 4 agents) End-stage renal disease(ESRD) 80 new patients/million population/y. Irreversible. Dialysis starts when GFR is 10–15% normal. Dialysis is needed lifelong unless a kidney transplant becomes available. Refer back to the renal unit managing the patient if you have any problems. Haemodialysis  Blood flows opposite dialysis fluid and substances are cleared along a concentration gradient across a semi-permeable membrane. Problems: pulmonary oedema; infection (HIV, hepatitis, bacteria); U&E imbalance; BP d or i; problems with vascular access; dialysis arthro­ pathy (especially shoulders and wrists); aluminium toxicity;time. Continuous ambulatory peritoneal dialysis(CAPD)  A permanent catheter is inserted into the peritoneum via a subcutaneous tunnel. 72L dialysis fluid is introduced and kept in the peritoneum. This is changed for fresh fluid up to 5x/d at home. Does not tie the patient to a dialysis machine. Problems: peritonitis; catheter blockage (refer as an emergency); weight i; poor DM control; pleural effusion; leakage. Anaemia and erythropoietin  s anaemia due to d kidney erythropoietin production is universal amongst people with ESRD. Exclude other causes. Recombinant erythropoietin is given if Hb 90

Kidney damage with normal or i GFR

3.3

i BP

Yearly

2

60–89

Kidney damage with mild d GFR

3

i BP

Yearly

3A

45–59

Moderate d GFR 4.3

3B

30–44

i BP, Ca2+ and 6mo PO4– changes, renal anaemia, LVF

4

15–29

Severe d GFR

0.2

As above + i K+ 3–6mo

5

50% progress to CKD

Membranoproliferative

50% present as nephrotic syndrome. Associations—endocarditis, C3 nephritic factor (autoantibody), hepatitis C, measles

Proliferative

Presents with nephritic syndrome. Classically seen 2wk after Strep. infection. Prognosis is excellent

IgA disease (Berger’s disease)

Causes recurrent haematuria in young men. Asimilar histological picture is seen in Henoch–Schönlein purpura (b p.526). 30% progress to ESRD

Rapidly progressive/ Presents with haematuria, oliguria, i BP, acute renal failure. crescentic Vigorous treatment may preserve renal function. Causes: anti-glomerular basem*nt membrane disease (Goodpasture’s disease), Wegener’s granulomatosis, Henoch–Schönlein purpura

Kidney diseases

Haemolytic uraemic syndrome  Most common cause of AKI in children. Usually follows gastroenteritis. Due to E.coli toxin. Have a high index of suspicion in any child with bloody diarrhoea. Occasionally occurs without diarrhoea. Other features: • Dehydration • CNS symptoms—irritability, • Oliguria (though may be polyuria) drowziness, ataxia,coma • Proteinuria/haematuria • i BP (associated with • Haematological features—anaemia, non-diarrhoeal disease) thrombocytopenia ± purpura Management  Admit for specialist care—often including dialysis. If associated with diarrhoeal illness, >80% make full recovery. Mortality is 1.8%. Poor prognostic indicators are age >5y at onset and dialysis for >2wk. Disease in the absence of diarrhoea has poorer prognosis. Adult polycystic kidney disease Autosomal dominant disease (1:1,000). Cysts develop in the kidney causing gradual d in renal function. Common cause of CKD. Presents with haematuria, UTI, abdominal mass (30% have cysts in the liver/pancreas too), lumbar/abdominal pain, and/ or i BP. May be associated with mitral valve prolapse and SAH/berry aneurysms. USS shows large kidneys with multiple cysts. Refer to a renal physician if CKD 3–5. Treat infections and i BP. Check family members (though cysts may not be seen 5. There may be a family history. Most are asymptomatic and the condition is an incidental finding. If symptomatic, presents with UTIs, renal stones, haematuria. Refer if symptomatic. Usually prognosis is very good and most require no treatment.

Renal vein thrombosis(RVT) Causes: nephrotic syndrome (15–20% develop RVT); membranous GN (30%); acute dehydration. Presentation varies from no symptoms to severe pain and loin tenderness. Suspect in at-risk individuals if unexplained loss of renal function and RBCs in urine. Refer to a renal physician for further investigation. Renal artery stenosis Causes: atheroma, fibromuscular hyperplasia (in the young). Presents with i BP (may be severe or drug-resistant); vascular disease elsewhere; abdominal bruit; i Cr, d eGFR, and proteinuria. If bilateral or extensive, renal failure may be precipitated by dehydration, dBP, or drugs (ACE/ARB initiation; NSAIDs). Refer to a renal physician (if diagnosis is unsure) or vascular surgeon (if diagnosis is known).

Alport’s syndrome X-linked or autosomal inherited disease. Congenital sensorineural deafness, haematuria, proteinuria, and renal failure. Associated with lens abnormalities, platelet dysfunction, and i BP. Causes ESRD by third decade in 4; 5 rarely develop ESRD. Renal failure does not recur after transplantation. A.C. Alport (1880–1959)—South African physician. Patient support and information UK National Kidney federation F 0845 601 02 09 M www.kidney.org.uk

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Renalstones 12% of 4 and 3% of 5 will develop a renal stone at some point; peak age 20–50y. Symptoms are not dependent on size of thestone.

Risk factors • Family history—i risk x3. Specific conditions: X-linked nephrolithiasis, cystinuria, hyperoxaluria • Anatomically abnormal kidneys, e.g. horseshoe kidney, medullary spongekidney • Metabolic disease, e.g. gout, hypercalcaemia/hypercalciuria, cystinuria, renal tubular acidosis or other acidosis (ileostomy, adenomatous polyp), oxaluria, aminoaciduria • Dehydration • Immobilization • ChronicUTI Drugs predisposing patients to stone formation  Acetazolamide, allopurinol, aspirin, steroids, indinavir, nelfinavir, loop diuretics, probenecid, quinolones, sulfonamides, theophylline, thiazides, triamterene, antacids, calcium/vitamin D supplements, high-dose vitaminC.

Presentation  Usually presents with pain ± nausea/vomiting. Location

and type of pain gives clues about the site of thestone: • Loin pain—kidneystone • Strangury—bladderstone • Renal colic—uretericstone • Interruption of flow—urethralstone Renalcolic • Symptoms Severe pain with waves of i severity. Usually starts abruptly as flank pain which then radiates around the abdomen to the groin as stone progresses down the ureter. May be referred to testis/ tip of penis in men or labia majora inwomen • Signs Patient is obviously in pain—usually unable to sit still and keeps shifting position to try to get comfortable (in contrast to peritonitis where patients tend to keep still). May be pale and sweaty. May be mild tenderness on deep abdominal palpation or loin tenderness, though often minimal signs. If fever suspect infection Other presentations UTI, haematuria, retention, renal failure (rare).

Differential diagnosis Pyelonephritis; ruptured AAA; cholecystitis; pancreatitis; appendicitis; diverticulitis; obstruction; strangulated hernia; testicular torsion; pethidine addiction.

Immediate investigations Dipstick urine if possible. Absence of RBCs does not exclude renal colic but consider alternative diagnosis. Immediate management Stones usually pass spontaneously. Give

pain relief (diclofenac admission to hospitalif: • Fever • Oliguria • Poor intake offluid

75mg IM/100mg PR) ± antiemetic. Consider

• Pregnant • Livesalone • Uncertain diagnosis

• Analgesia ineffective / short-lived • Symptoms>24h

If not admitted Encourage i fluid intake; sieve urine for stones. Monitor/review pain relief and for complications.

Renalstones

Further investigations  Can wait until the next working day and include: • Blood U&E, creatinine, eGFR, Ca2+, PO43–, alkaline phosphatase, uric acid, albumin • Urine M,C&S; RBCs. Consider checking ‘spot’ test for urine cystine, and TPCR, Ca2+, PO43–, uric acid, and sodium excretion • Radiology X-ray of kidneys, ureters, and bladder—90% of renal stones are radio-opaque—only urate and xanthine stones are radio-transluscent; renal tractUSS

Follow-up  50% recur in 5–7y. Give general advice on prevention of

stones (see Table14.6). If investigations show any loss of renal function, renal obstruction, or remaining stones—refer to urology. Dependent on composition of stones, give dietary advice/refer to dietician (see Table14.6).

Hyperoxaluria  May be 1° (autosomal recessive condition) or s to gut resection/malabsorption or dietary excess of spinach or vitamin C.Take specialist advice on management. There are two types of p hyperoxaluria: • Type 1 hyperoxaluria Calcium oxalate stones are widely distributed throughout the body. Presents as renal stones and nephrocalcinosis in children. 80% have chronic renal failure in2–2.5L/24h), especially in hot weather; d weight if obese; d animal protein and i fruit/ vegetables in diet; d salt intake

Calcium oxalate

Urinary alkalinization with potassium citrate; avoid chocolate, tea, rhubarb and spinach, nuts, beans, beetroot; d citrus fruits; bendroflumethiazide 2.5mg od may help if hypercalciuria; hyperoxaluria is treated with pyridoxine

Calcium phosphate

Low Ca2+ diet; avoid vitamin D supplements. Bendroflumethiazide 2.5mg od may help if hypercalciuria

Staghorn/triple phosphate Associated with UTI due to Proteus species and (calcium, magnesium, and urinary stasis, e.g. due to anatomical abnormality. Treat UTI with antibiotics ammonium) Urate

Avoid beer as has uricosuric effect; allopurinol; urinary alkalinization with potassium citrate (pH >6.5)

Cystine

Urinary alkalinization with potassium citrate

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Haematuria, bladder and renalcancer Haematuria  Blood in the urine. Causes—see Table14.7.

• May be frank (visible) or microscopic (up to 20% population). • Investigate all cases of haematuria further • Check MSU for M,C&S, and blood for U&E, creatinine, and eGFR. Free Hb and myoglobin make urine test sticks +ve in absence of redcells • Urine discoloration can result from beetroot ingestion, porphyria, or rifampicin • If cause is identified (e.g. sample taken when menstruating, UTI)— repeat the check for blood in urine once treated/resolved • Refer if no cause is found. Rapid access one-stop clinics are now operated in mostareas Table14.7  Causes of haematuria Kidney

Stones Tumour

Infection Glomerulonephritis

Ureter

Stones

Tumour (rare)

Bladder

UTI Stones

Tumour Chronic inflammation

Prostate

Prostatitis

Tumour

Urethral inflammation

Sterilepyuria  Presence of white cells in the urine in the absence of UTI. Causes: • Interstitial nephritis or • Bladdertumour • Inadequately cystitis • RenalTB treatedUTI • Polycystickidney • Papillary necrosis • Appendicitis • UTI with failure to • Chemical cystitis, e.g. • Calculi due to radiotherapy culture organism • Prostatitis Management  Initially repeat with clean-catch MSU. If finding persists refer to urology. Management of haematuriaN Urgent referral  Patients: • Of any age with painless macroscopic haematuria • Aged ≥40y with recurrent/persistent UTI associated with haematuria • Aged ≥50y with unexplained microscopic haematuria • With an abdominal mass identified clinically or on imaging that is thought to arise from the urinarytract Non-urgent referral Patients 40y and 4) and/or radiology (e.g. renal tract USS, KUB)if: • Unclear diagnosis (e.g. persisting • UTI inaman symptoms but negativeMSU) • UTI in a child (b p.879) • Unusual infecting organism • Recurrent UTI in awoman • Sterile pyuria (b p.446) • Pyelonephritis

Management Catheterized patients—bp.453    Pregnantwomen—bp.812 Children—bp.878 All other patients • i fluid intake (>3L/24h). Alkalinize urine (e.g. potassium citrate solution) to ease symptoms • Prescribe oral antibiotics, e.g. trimethoprim 200mg bd (80% organisms are sensitive). Use a 3d course for women with uncomplicated UTI, a 7d course for men, patients with GU malformations or immunosuppression, relapse (same organism) or recurrent UTI (different organism). Use a 7d course of a quinolone (e.g. ciprofloxacin 500mg bd) for patents with pyelonephritis • Refer to urology if any abnormalities are detected on further investigation or unable to resolve symptoms. Admission is rarely needed Prevention of recurrent cystitis  Reinfection after successful treatment of infection (90%) or relapse after inadequate treatment. • General advice Advise patients to urinate frequently; i fluid intake; double void (i.e. go again after 5–10min) and void after intercourse. X Efficacy of cranberry juice is controversial • Prophylactic antibiotics Consider prescribing either post-coitally (e.g. nitrofurantoin 50mg stat) or continuously (trimethoprim 100mg nocte or nitrofurantoin 50mg nocte) • Men with BPH Finasteride or dutasteride and/or doxazosin d incidenceofUTI • HRT Topical oestrogen d recurrent UTI in women of allagesR • Vaccines Results of large-scale trials are awaited Prostatitis  b p.456     Chronic pyelonephritis bp.442 Urethral syndrome  Symptoms of cystitis with–ve MSU. Unknown cause. Associated with cold, stress, nylon underwear, CHC, and intercourse. Advise fluids ++ and to wear cotton underwear. Consider changing/stopping CHC or trying topical oestrogen if post-menopausal. Tetracyclines (e.g doxycycline 100mg bd for 14d) or azithromycin (500mg od for 6d)R are helpful in some. If not settling, refer to urology. Urethral dilatation/massage may be helpful. Interstitial cystitis Predominantly middle-aged women. Can cause fibrosis of the bladder wall. Main symptoms—frequency, urgency, and pelvic/suprapubic pain especially when the bladder is full. Often mis­diagnosed as recurrent UTI. MSU—no bacteriuria. Refer to urologist for confirmation. There is no satisfactory treatment, though antispasmodics, amitriptyline, and bladder stretching under GA may help some patients.

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Incontinence ofurine Involuntary loss of urine which is objectively demonstrable and a social or hygienic problem. 1 in 3 with incontinence consult at outset, 1 in 3 consult later, 1 in 3 suffer in silence. Opportunistic questioning can identify sufferers.

History • Frequency of complaint • Volumepassed • Degree of incapacity • Whether occurs with standing/ coughing/sneezing

• Urgency/dysuria/frequency of micturition • Past obstetric and medical history • Medication • Mobility and accessibility of toilets

Examination • Abdominal including DRE—enlarged bladder, masses, loaded colon, faecal impaction, analtone • Pelvic—prolapse, atrophy, neurological deficit, retention of urine, and pelvic masses

Investigation  Intake/output diary (at least 3d, including working and leisure days)—evaluates problem and benchmark for progress—record drinks and passage of urine; urine—RBCs, MC&S; consider blood for U&E, eGFR, FBC, FBG/HbA1c if renal impairment/DM is suspected.

Drugs that exacerbate/cause incontinence Diuretics, antihistamines, anxiolytics, α-blockers, sedatives and hypnotics, anticholinergic drugs,TCAs. GP management  0 30% have a mixed pattern. Treat according to dominant symptom. Try general measures before referring to urology/ gynaecology or for further investigations (see Table14.9). General measures • Manipulate fluid intake:amount, type (avoid tea, coffee, alcohol), timing • Promote weightd • Alter medication, e.g. timing of diuretics • Treat UTI and chronic respiratory conditions • Avoid constipation • Consider HRT (topical or systemic) for oestrogen deficiency • Consider scheduled voiding if cognitive deficit Aids and appliances  bp.452 Nocturnal enuresis in children  bp.914 Stress incontinence • Symptoms Small losses of urine without warning throughout the day related to coughing/exercise • Causes Prostatectomy; childbirth; deterioration of pelvic floor muscles/ nerves • Treatment Pelvic floor exercises (b p.841) continued >3mo help 60% (taught by physiotherapists/continence advisors; leaflets available)—may be assisted by vagin*l cones and/or electrical stimulation. Mechanical devices (e.g. Contrelle Activguard®, FemSoft®) mayhelp.

Incontinence ofurine

Urge incontinence (overactive bladder syndrome)  Detrusor instability or hyperreflexia cause the bladder to contract unintentionally • Symptoms Frequency, overwhelming desire to void (often precipitated by stressful event), large loss, nocturia • Causes Idiopathic, neurological problems (stroke, MS, DM, spinal cord injury, dementia, PD), local irritation (bladder stones, bladder cancer, infection), obstruction (BPH), surgery (TURP) • Treatment Bladder training—resist the urge to pass urine for i periods. Start with an achievable interval based on diary evidence and i slowly—continue for >6wk. If bladder training is ineffective, try oxybutinin first-lineN (alternatives:darifenacin, solifenacin, tolteridone, trospium). Spontaneously remits/relapses, so reassess every3–4mo Overflow  Constant dribbling loss day and night. Causes: BPH, prostate cancer, urethral stricture, faecal impaction, neurological (LMN lesions), side effect of medication. Treatment is aimed at relieving the obstruction (b p.454). Urinary fistula  Communication between bladder and the outside—normally through the vagin*. Results in constant dribbling loss day and night. Refer to gynaecology/urology. Causes: congenital, malignancy, complication of surgery. Functional incontinence  No urological problem. Caused by other factors, e.g. inaccessible toilets/immobility, behavioural problems, cognitive deficit. Treat thecause. Table14.9  Referral for incontinence problems Specialist continence advisor/DN

•  Advice on aids or appliances •  Advice on primary care management •  Patient support

Urodynamic studies

•  If type of incontinence is uncertain •  Atypical features of incontinence •  After unsuccessful surgery •  If a neurological problem is suspected

Gynaecology or urology opinion

•  GP management hasfailed •  Severe symptoms and/orpain •  RecurrentUTI •  Concomitant gynaecological problems (e.g. prolapse) •  Concomitant urological problems (e.g. chronic retention, prostate abnormality on rectal examination) •  Failed incontinence surgery •  Pelvic radiotherapy •  Vesico-vagin*l fistula • Haematuria b p.446

Further information European Association of Urology Guidelines on urinary incontinence (2012) M www.uroweb.org NICE Urinary incontinence (2006) M www.nice.org.uk

Patient information and support Bladder and Bowel Foundation F 0845 345 0165 M www.bladderandbowelfoundation.org

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Aids and appliances for incontinence Pads  Many different types. DNs or continence advisors are best aware of those available via the NHS locally. They are not available on FP10 and supplied by local NHS Trusts on a ‘daily allowance’ basis. This varies across the country.

Bedcovers  Absorb 1–4L of urine. Good laundry facilities are needed. If left wet can cause skin breakdown. Available via NHS Trusts. Sheaths or external catheters  Can be prescribed on NHS prescrip-

tion. Approved appliances are listed in part IXB of the UK Drug Tariff. Used for men who have intractable incontinence and who are highly physically dependent, do not have urine retention and do not require an internal catheter. Assessment and fitting by a DN or continence adviser is essential. Used in association with a drainage bag. Sheaths may be non-adhesive, self-adhesive or attached with adhesive strips. Adhesive sheaths can last several days but daily changing is recommended. Replace non-adhesive sheaths 2–3x/d (some are reusable). Problems  Include i susceptibility to UTI, sores on penis, and skin irritation due to the adhesive.

Catheters  Can be prescribed on NHS prescription. Approved appliances are listed in part IXA of the UK Drug Tariff.

Indwelling catheters  Only use catheters in patients whohave: • Urinary retention or neurogenic bladder dysfunction • Severe pressuresores • Inoperable obstructions that prevent the bladder emptying • Terminal illness • Housebound without adequate carer support

Types  Only long-term Foley catheters are suitable for use in primary care. They last 3–12wk. Cathetersize Unless specified a 12 or 14Ch catheter is supplied. Use the smallest diameter of catheter that drains urine effectively. Catheters >16Ch are more likely to cause bypassing of urine around the catheter and urethral strictures. Catheterlength  Men require longer catheters than women. Specify ‘male’ or ‘female’ on the prescription. Catheter balloon  10mL balloons are supplied unless specified otherwise. Pre-filled catheters contain sterile water which inflates the retaining balloon with water. They are more expensive but quicker to insert and there are no costs for syringes or sterilewater. Insertion  bp.455 Drainage  Usually attached to a leg bag, although catheter valves are also available allowing the patient to use his/her bladder as a urine reservoir. The valve must be released every 3–4h to drain out theurine.

Aids and appliances for incontinence

Common problems • Leakage Check no constipation, check catheter not blocked, try smaller gauge catheter • Infection 90% develop bacteriuria 7.4, or a daily dose of vitaminC • Inflammation Results from physical presence of a catheter in the urethra. Exacerbated by encrustation and infection. There is no easy solution—try a different brand catheter (e.g. hydrogel catheter rather than silicone) • Blockage Change catheter. The interval of routine changes should be altered if there is regular blockage towards the end of the life of a catheter

Intermittent self-catheterization Patient inserts a catheter into

his/her bladder 4–5x/d to drain urine. d problems of infection and blockage. Useful for neurological bladder dysfunction.Types: • Reusable silver or stainlesssteel • Reusable PVC—washed and reused for 1wk. Usually supply5/mo • Single use—need 125–150/mo. Expensive. Only use on consultant advice

Collectingbags  Can be prescribed on NHS prescription. Approved appliances are listed in part IXB of the UK Drug Tariff. • Leg bags Drainable bags last 5–7d. Usually 500/750mL. Larger capacity bags are too heavy for mobile patients. Avariety of attachment systems are available on prescription. Long tubes are needed to wear a bag on thecalf • Night drainage bags Connect to night bag attachment of day bags Single use, disposable non-draining bags are recommended. Bag hangers are not available on NHS prescription

Enuresisalarms  bp.915 Further information NHSBSA Electronic drug tariff M www.nhsbsa.nhs.uk/prescriptions

Patient advice and support Bladder and Bowel Foundation F 0845 345 0165 M www.bladderandbowelfoundation.org

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Urinary tract obstruction Causes of obstruction  (See Figure14.1) Obstruction may be unilateral

(kidney, pelvi-ureteric junction, or ureter) or bilateral (bladder, urethra, prostate). Unilateral obstruction may present late if the other kidney remains functioning. Suspect if loin ache worsened by drinking. Confirm with USS and refer to urology. Obstructing lesions may be in the lumen (e.g. stones) in the wall (e.g. tumours) or impinging from outside (e.g. retroperitoneal fibrosis).

Acute retention ofurine Sudden inability to pass urine l lower abdominal discomfort with inability to keep still. Differentiate from AKI. 4> 5. Risk factors: age >70y; symptoms of prostatism/poor urinary stream. Causes  Prostatic obstruction (82%); constipation; alcohol; drugs (anticholinergics, diuretics); UTI; operation (e.g. hernia repair). Rarer causes: urethral stricture; clot retention; spinal cord compression; bladderstone. Examination  Abdomen—palpable bladder; DRE—enlarged ± irregular prostate; perineal sensation to exclude neurologicalcause. Investigation  MSU to exclude infection. Blood for U&E, Cr, and eGFR. Only investigate if catheterizing in the community. Management  Catheterize (record initial volume drained) or refer to urology for catheterization—local policies vary. Treat infection. Refer to DN for instruction on management of the catheter. Refer to urology for further assessment and treatment.

Chronic retention ofurine  Insidious onset. Causes: benign prostatic hypertrophy; pelvic malignancy; CNS disease. May presentas: • Nocturnal enuresis • Acute on chronic retention • UTI • Overflow incontinence • Lower abdominalmass • Renal failure Examination and investigation  As for acute retention. Bladder is enlarged (may contain >1.5L) but usually non-tender. Management  Refer to urology for further assessment and treatment. Refer urgently or acutely if pain, UTI or renal failure (eGFR 3mo historyof: • Urological pain—lower abdomen, pelvis/perineum, penis (especially tip ± on ejacul*tion), testicl*s, rectum, lowback± • Irritative/obstructive symptoms and/or ejacul*tory disturbance Diagnosis is based on history with exclusion of other causes. Suitable investigations include DRE, MSU, urine cytology, STI screen (b p.739), PSA ± urodynamic studies. Treatment is difficult—provide information and support; try α-blockers (e.g. doxazosin 4mg od for 6mo). Spontaneous improvement/remission often occurs.

Patient support Prostatitis Foundation M www.prostatitis.org

Further information BASHH Management of prostatitis (2008) M www.bashh.org.uk Cochrane Tacklind J, MacDonald R, Rutks I, et al. (2012) Serenoa repens for benign prostatic hyperplasia. Cochrane Database of Systematic Reviews, Issue 12. Art. No.: CD001423. DOI: 10.1002/14651858.CD001423.pub3.

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More than half the time

Almost always

Your score

1

2

3

4

5

Over the past month, how often have you had to urinate again 75y); very few become clinically evident, so many more men would be found with prostate cancer by screening than would die or have symptoms fromit • Natural history of prostate cancer is not understood—there is no means to detect which ‘early’ cancers become more widespread • Inadequate screeningtests • It is not clear if early treatment enhances life expectancy • Peak incidence of morbidity and mortality is in old age (75–79y), so potential years of life saved by screening aresmall Screeningtests • Prostate-specific antigen (PSA) bp.459 • Digital rectal examination (DRE) Operator-dependent, fails to detect early prostate cancers, and lacks specificity. Annual screening in the USA and Germany has not d mortality • Transrectal ultrasound (TRUS) Too expensive The most effective screening regime involves rectal examination and PSA testing followed by TRUS for suspicious lesionsS. Optimal screening interval is unknown but serial screening does i detection.

Symptoms andsigns Earlycancer  Symptomless. Usually detected following an incidental finding of i PSA. Hard nodule sometimes felt in prostate onDRE.

Prostatecancer

Local disease • Prostatism • Urinary retention • Haematuria

• Lower extremityoedema • On rectal examination, the prostate is hard and non-tender and sulci lose definition

Metastatic disease • Malaise • Weightloss • Bonepain • Pathological fractures

• Spinal cord compression • Ureteric obstruction may cause renal failure • Signs depend on site of metastases

InvestigationN  A digital rectal examination and PSA test (after counselling) are recommended for patients with any of the following unexplained symptoms: • Inflammatory or obstructive • Erectile dysfunction lower urinary tract symptoms • Haematuria • Weight loss, especially in the • Lower backpain elderly • Bonepain 0 Exclude UTI before PSA testing and postpone digital rectal examination until after the PSA test isdone.

Urgent referralN • Rectal examination—hard, irregular prostate typical of prostate cancer. PSA result should accompany the referral • Rectal examination—normal prostate, but rising/raised age-specific PSA ± lower urinary tract symptoms* • Symptoms and high PSA levels • Asymptomatic men with borderline, age-specific PSA results repeat PSA after 1–3mo. If the PSA level is rising, refer the patient urgently *  Consider discussion with specialist and patient ± carer before referral for very elderly patients/those compromised by other co-morbidities.

0 Referral is not needed if the prostate is simply enlarged and the PSA is in the age-specific referencerange.

Further information forGPs NICE M www.nice.org.uk • Referral guidelines for suspected cancer(2005) • Prostate cancer:diagnosis and treatment(2008) Cancer research UK M www.cancerresearchuk.org National screening M www.cancerscreening.nhs.uk

Information for patients on PSA testing and prostatecancer National screening M www.cancerscreening.nhs.uk Cancer Research UK F 0808 800 4040 M www.cancerhelp.org.uk Macmillan Cancer Support F 0808 808 0000 M www.macmillan.org.uk Prostate Cancer Charity F 0800 074 8383 M www.prostatecancer.org.uk Prostate Cancer Support Association F 0845 601 0766 M www.prostatecancersupport.co.uk

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Treatment of prostatecancer Symptomless local disease  Treatment is controversial. There are

two arguments:

Benefits of treatment are outweighed by risks

or

Aggressive treatment before spread is the only way to ensure cure

>50% of men >50y who die from other causes are found post-mortem to have prostate cancer—prostate cancer kills only a small minority of men who have it. The personal and economic cost of treating men whose cancer would never have caused them any problems must be considered. Options • Watchful waiting or active surveillance Monitor with PSA and regular rectal examination. i in PSA or size of nodule triggers active treatment. At 10y follow-up 100:metastatic spread is verylikely Prognosis  5y survival rates for tumourstage: • 1 or 2—tumour confined within the prostate (65–98%) • 3—tumour has breached the capsule of the prostate(60%) • 4—spread to LNs, within the pelvis or elsewhere (20–30%)

Further information NICE Prostate cancer:diagnosis and treatment (2008) M www.nice.org.uk Cancer Research UK M www.cancerresearchuk.org National screening M www.cancerscreening.nhs.uk

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Conditions of thepenis Posterior urethralvalves Folds of mucosa inhibit or block passage of urine causing urethral, bladder, ureter, and renal pelvis dilatation. Presentation  Usually detected on antenatal USS. Can present in neonates with urinary retention or dribbling urine + distended bladder, UTI or uraemia, or later in childhood with recurrent UTI or incontinence. Investigation and management  MCUG confirms diagnosis. In all cases refer to urology for surgical disruption of the valves.

Hypospadias  1 in 400 male births. The urethral meatus opens on the ventral side of the penis. There is often hooding of the foreskin and ventral flexion of the penis. Refer to urology. Treated with corrective surgery, ideally preschool. Non-retractile foreskin  Usually noted by parents. May be history of recurrent balanitis. Examination: foreskin adherent. Management  Age 4y and/or recurrent balanitis, consider treatment with topical steroids (e.g. betamethasone 0.1% od) for 3–4mo. If ineffective, refer to paediatric surgery for circumcision.

Phimosis  Foreskin obstructs urine flow. Common in small children. Time usually obviates the need for circumcision. Treat as for non-retractile foreskin if recurrent balanitis. Peyronie’s disease  Hard lumps in the shaft of the penis. Unknown

cause. 4% 4 >40y. 1 in 3 have pain/bending of the penis when erect. Associated with erectile dysfunction (b p.776). 5% have Dupuytren’s contracture. F.G.de la Peyronie (1678–1747)—French surgeon. Management  Reassurance usually suffices. No proven medical treatments. Refer to urology for surgery if pain or severe bending on erection so that intercourse is not possible.

Paraphimosis  Foreskin is retracted then (because of oedema) unable to

be replaced. Commonly occurs in catheterized patients when the catheter is changed.

Management  Try to replace foreskin using ice packs (d swelling) and lubrication (e.g. KY jelly). If unable to replace the foreskin, admit for surgery.

Balanitis  Acute inflammation of glans and foreskin. Common organisms—staphylococci, streptococci, coliforms, candida. Can occur at any age. Most common in young boys when associated with non-retractile foreskin/phimosis. In elderly patients considerDM. Management  Oral antibiotics (e.g. flucloxacillin) or topical antifungals (e.g. clotrimazole). If recurrent or secondary to phimosis consider referral for circumcision.

Conditions of thepenis

Balanitis xerotica et obliterans  Chronic fibrosing condition of the foreskin which may become adherent to the glans. Treatment is with topical steroid creams, e.g betamethasone 0.1%. Consider referral for circumcision.

Trauma to the foreskin  Torn frenulum—seen after poorly lubricated intercourse or if caught in a zip. No treatment required. If recurrent, consider referral for circumcision. Erectile dysfunction  bp.776 Priapism  Persistent painful erection not related to sexual desire. Cause  Medication for erectile dysfunction, idiopathic, leukaemia, sickle cell disease, or pelvic tumour. Management  Ask the patient to climb stairs (arterial ‘steal’ phenomenon), apply ice packs. If unsuccessful refer to A&E for aspiration of corpora. Rarely surgery is needed.

Erythroplasia of Queryat  Pre-malignant condition of glans. Moist velvety-looking patches. Refer to urology. Treatment is surgical. Carcinoma of thepenis  Squamous cell carcinoma (95%) or malignant melanoma. Usually elderly men. Rare in theUK. ManagementN  Refer urgently patients with symptoms or signs of penile cancer. These include: • Progressive ulceration in the glans, prepuce, or skin of the penileshaft • Mass in the glans, prepuce, or skin of the penileshaft 0 Lumps within the corpora cavernosa can indicate Peyronie’s disease, which does not require urgent referral.

Penile discharge  Associated with urethritis, e.g. due to chlamydia or gonorrhoea. Refer to GUM clinic.

Further information BASHH Management of balanitis (2008) M www.bashh.org.uk NICE Referral guidelines for suspected cancer (2005) M www.nice.org.uk

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Renal medicine and urology

Testicular disease Testicularpain  Treat thecause:

  • Varicocele • Epididymo-orchitis   • Testicular tumour (rarely • Torsion of thetestis painful) • Trauma and haematoma formation

Torsion of thetestis  Peak age 15–30y. Presents with sudden onset of severe scrotal pain. May be associated with right iliac fossa pain, nausea, and vomiting. Examination: tender, hard testis riding higher than contralateral testis. Admit urgently to surgical/urologyteam. Torsion of the hydatid of Morgagni  Small embryological remnant at the upper pole of the testis. Presents similarly to torsion of the testis. Refer as an emergency to exclude torsion of the testis.

Epididymo-orchitis  Inflammation of the testis and epididymis due to infection. May occur at any age. The most common viral cause is mumps. The most common bacterial causes are chlamydia or gonococci (35y). Chronic infection with TB or syphilis israre. Presentation  Acute onset pain in testis; swelling and tenderness of testis/ epididymis; fever ± rigors; may be urethritis, dysuria, and/or i frequency. Management  May be difficult to distinguish from torsion of the testis. If in doubt, admit for urology/surgical opinion. Otherwise investigate and treat for the underlyingcause. Testicular lumps and swellings  See Figure14.2. Hydrocele  Collection of fluid in the tunica vagin*lis. Occurs at anyage. • p hydrocele—no predisposing cause in scrotum • s hydrocele—reaction to pathology in testis or covering (infection, tumour, torsion). In adults presenting with hydrocele always consider impalpable tumour beneath Presentation  Swelling in the scrotum. The examiner should be able to get above the swelling. Smooth surface, transilluminates; testis is within the swelling and not palpable separately. Management  Investigation is not required in children; refer adults for USS if testis is not palpable. Options for adults: • Conservative management—reassurance; small hydroceles • Tapping—may be suitable for large hydroceles where surgery is inappropriate; s infection and recurrence arecommon • Surgery—refer to urologist Hydroceles in children are usually congenital. May be unilateral or bilateral. Most resolve spontaneously in the first year oflife. Refer to urology if persists>1y.

Hydrocele of thecord  Arises in part of the processus vagin*lis in the

spermatic cord above the testis. Rounded lump which slips up and down the inguinal canal. No action needed.

Testicular disease

Can you get above the mass?

YES

Is it cystic?

1 Any acutely painful scrotum should be treated as a torsion of the testis until proven otherwise

NO

Differential diagnosis: • Inguinal hernia extending into the scrotum • Varicocele • Hydrocele of the spermatic cord NO—solid mass

YES

Is it separate from the testis?

Is it separate from the testis?

YES

NO—the testis lies within the swelling

YES

NO

Differential diagnosis: • Acute or chronic epididymitis • Torsion of hydatid of Morgagni

Differential diagnosis: • Epididymal cyst • Spermatocele Differential diagnosis: • Hydrocele (transilluminates) • Haematocele

Differential diagnosis: • Tumour • Torsion

• Orchitis • Gumma

Figure14.2  Diagnosis of testicularlumps

Referral guidelinesN • Refer urgently patients with a swelling or mass in the body of the testis • Consider an urgent ultrasound in men with a scrotal mass that does not transilluminate and/or when the body of the testis cannot be distinguished

Further information NICE Referral guidelines for suspected cancer (2005) M www.nice.org.uk BASHH Management of epididymo-orchitis (2010) M www.bashh.org.uk

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Haematocele  Damage to the testis (e.g. due to a direct blow, vasectomy) can result in the testis rupturing and the tunica vagin*lis filling with blood. Refer as an emergency for urological assessment. Varicocele  Collection of varicose veins in the pampiniform plexus of the cord and scrotum. Can be s to obstruction of the testicular veins in the abdomen. L > R.Associated with infertility (thought due to i temperature of testis). Presents with a dull ache in the testis especially at the end of the day or after exercise. Usually visible when the patient is standing. No treatment is needed—reassure. Occasionally surgery or radiological embolization may help if symptoms are severe. Epididymalcyst  Common and often multiple. Found in middle-aged/ elderly men. Usually presents when the patient finds a painlesslump. • Examination Smooth-walled cyst in epididymis (palpable above and behind testis), often bilateral • Investigation If unsure of diagnosis refer forUSS • Management Reassurance. Refer to urology if painful Spermatocele  Cyst containing sperm. Typically situated in the head of the epididymis—more rarely in the spermatic cord. Clinically presents in the same way as epididymal cyst. Management is thesame. Testiculargumma  bp.749 Benign testicular tumours  Rare (40y) and usually asymptomatic. Pain is generally intermittent and related to activity. Examination reveals d neck mobility. Severe degeneration can cause nerve root signs. Treat with analgesia ± cervical collar. X-ray only if conservative measures fail, troublesome pain, nerve root signs, or the patient has psoriasis (? psoriatic arthropathy). Nerve root irritation or entrapment  Secondary to degeneration, vertebral displacement/collapse, disc prolapse, local tumour, or abscess. Causes neck stiffness, pain in arms or fingers, d reflexes, sensory loss, and d power. The level of entrapment can usually be determined clinically (see Table 15.1). Treat with analgesia ± cervical collar. X-ray cervical spine—lateral or oblique views. Refer for physiotherapy. Refer for further investigations (e.g. MRI) if conservative management fails and there is objective evidence of a root lesion.

Neckpain

Table15.1  Neurology associated with cervical nerve root entrapment Root

Sensory changes

Motor weakness

C5

Lateral arm

Shoulder abduction/flexion Biceps Elbow flexion

Reflex changes

C6

Lateral forearm Thumb Index finger

Elbow flexion Wrist extension

Biceps Supinator

C7

Middle finger

Elbow extension Wrist flexion Finger extension

Triceps

C8

Medial side of lower forearm Ring and little fingers

Finger flexion

None

T1

Medial side of upper forearm

Finger abduction/ adduction

None

• Refer urgently if there are signs of spinal cord compression: • Root pain and lower motor neurone signs at the level of the lesion,and • Spastic weakness, brisk reflexes, upgoing plantars, loss of coordination and sensation below the lesion Spasmodic torticollis (wry neck)  Common. Sudden onset of painful stiff neck due to spasm of trapezius and sternocleidomastoid muscles. Self-limiting. Heat, gentle mobilization, muscle relaxants, and analgesia can speed recovery. Acervical collar may help in the short term but can prolong symptoms. Often caused by poor posture, e.g. computer-seating position; carrying heavy, unevenloads. Cervical rib Congenital condition of C7 vertebra costal process enlargement. Usually asymptomatic but can cause thoracic outlet compression l hand or forearm pain, weakness or numbness, and thenar or hypothenar wasting. Radial pulse may be weak. X-ray of thoracic outlet may show cervical rib—but symptoms are sometimes due to fibrous bands that are not seen on X-ray. Refer to upper limb orthopaedic surgeon for further assessment. Whiplash injuries Neck pain resulting from stretching or tearing of cervical muscles and ligaments due to sudden extension of the neck—often due to a RTA. Pain and d neck mobility typically starts several hours or days after injury. Pain may radiate to shoulders, arms, andhead. Management  Examine carefully to exclude bony tenderness requiring X-ray. Treat with analgesia and early mobilization—collar may help initially but avoid long-term use. Recovery is often slow and 40% patients suffer long-lasting symptoms. As a general rule of thumb, the quicker the symptoms develop, the longer they will take to disappear. Early physiotherapy, if available, can improve recovery rate. Psychological problems and medicolegal issues can affect progress.

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Low backpain Definitions • Acute low back pain New episode of low back pain of 3mo Causes of back pain  See Table15.2. History Ask: • Circ*mstances of pain—history of injury; duration • Nature/severity of pain—pain/stiffness mainly at rest/at night, easing with movement suggests inflammation, e.g. discitis, spondylarthropathy • Associated symptoms—numbness, weakness, bowel/bladder symptoms • PMH—past illnesses (e.g. cancer), previous back problems • Exclude pain not coming from the back (e.g. GI or GUpain)

Examination • Deformity, e.g. kyphosis (typical of ankylosing spondylitis), loss of lumbar lordosis (common in acute mechanical back pain), scoliosis • Palpate for tenderness, step deformity, and musclespasm • Assess flexion, extension, lateral flexion, and rotation whilst standing • Ask to lie down—this gives a good indication of severity of symptoms • In lower limbs look for muscle wasting and check power, sensory loss, and reflexes (knee jerk and ankle jerk). See Table 15.3. Assess straight leg raise (SLR)—sciatica is present if SLR on one side elicits back/buttock pain (usually ipsilateral but can be either side) compared to SLR on the otherside

• ‘Redflags’

• Past history of • 55y • Non-mechanicalpain cancer • Pain that worsens • HIV • Immune suppression whensupine • IV druguse • Night-timepain • Taking steroids • Thoracicpain

• Unwell • Weightd • Widespread neurology (see Table15.3) • Structural deformity

Management of acute pain in the community  Triage according

to history and examination—see Figure 15.1, b p.479.

For patients who do not require immediate referral  Prescribe analgesia, e.g. paracetamol ± NSAIDs ± amitriptyline (10–25mg nocte) and use the Keele STarT back screening tool (see Box15.1): • If total score ≤3, explain likely natural history of the pain and advise to avoid bed rest and maintain normal activities as far as possible (d chance of chronic pain). Suggest self-help exercises • If total score is ≥4, check question 5–9 sub-score: • If ≤3—if not resolved in 4wk, refer for physical therapy. Options include:back exercise classes, physiotherapy, chiropractic, osteopathy, or acupuncture, if available. • If ≥4—if not resolved in 4wk, refer directly for specialist intervention, sooner if worsening or severepain • In all cases, challenge any ‘yellow flag’ factors (see Figure 15.1, b p.479) that may inhibit recovery and delay return to normal functioning

Low backpain

Table15.2  Causes of back pain:age suggests the most likelycause Age (y)

Causes

15–30

• Postural • Mechanical •  Prolapsed disc

• Trauma • Fracture •  Ankylosing spondylosis

• Spondylolisthesis • Pregnancy

30–50

• Postural •  Prolapsed disc

• Spondylarthropathies • Discitis

• Degenerative joint disease

>50

• Postural • Degenerative •  Paget’s disease

• Malignancy (lung, breast, prostate, thyroid, kidney)

• Osteoporotic collapse • Myeloma

Other causes

•  Referredpain •  Spinal stenosis

• Cauda equina tumours

•  Spinal infection

Table15.3  Neurology with lumbosacral nerve root entrapment Root

Sensory changes

Motor weakness

Reflex changes

L2

Front of thigh

Hip flexion/adduction

None

L3

Inner thigh

Knee extension

Knee

L4

Inner shin

Knee extension Foot dorsiflexion

Knee

L5

Outer shin Dorsum of foot

Knee flexion Foot inversion Big toe dorsiflexion

None

S1

Lateral side of foot/sole

Knee flexion Foot plantarflexion

Ankle

Box15.1  Keele STarT Back Pain ScoringTool Ask patients to consider the following statements and state whether they agree or disagree with them. Thinking about the past2wk: 1. My back pain has spread down my leg(s) at some time in the last2wk 2. Ihave had pain in the shoulder or neck at some time in the last2wk 3. Ihave only walked short distances because of my backpain 4. In the last 2wk, Ihave dressed more slowly than usual because of backpain 5. It’s not really safe for a person with a condition like mine to be physically active 6. Worrying thoughts have been going through my mind a lot of thetime 7. Ifeel that my back pain is terrible and it’s never going to get any better 8. In general Ihave not enjoyed all the things Iused to enjoy If the patient agrees with a statement, score 1; if disagrees, score0. 9. Overall, how bothersome has your back pain been in the last2wk? • Not at all, slightly, or moderately—score0 • Very much or extremely—score 1

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0 Do not X-ray for back pain routinely  X-rays require a high radiation dose, and clinically meaningful findings are rare. Exceptions: • Young (80o from the vertical); crepitation? • Neurology Check for ulnar and median nerve function Hand • Look Posture of the hand; swellings (rheumatoid nodules; Heberden’s and Bouchard’s nodes; ganglions; tophi); nail signs, e.g. pitting of psoriasis; scars; deformity (mallet finger; swan neck deformity; Boutonnière deformity; Dupuytren’s contracture); ulnar deviation. If there is joint disease note distribution and whether it is symmetrical • Feel Temperature; condition of the skin, e.g. dryness, sweating; nature of swellings; muscle bulk, e.g. small muscles of the hand; tenderness • Move/measure Ask the patient to make a fist, spread his fingers out, and then test each individual joint. Then test opposition, pinch grip, key grip, palmar grasp of ball, and practical tasks, e.g. picking up acoin

Fractures  b p.1111. Ganglion Smooth, firm, painless swelling—usually around the wrist. No treatment is needed unless causing local problems. May resolve spontaneously; can be drained (large-bore needle)/excised but often recurs.

Wrist and hand problems

For all hand injuries  Checkfor: Nerve injury  Can occur due to trauma or lacerations of the hand or wrist. Examine sensory and motor function. Always ensure no other structures are damaged before suturing skin wounds. Refer all nerve injuries for specialist assessment and management—surgery can improve the outcome considerably in some cases. Intensive hand physiotherapy is important to regain function. Types of nerve injury: • Neurapraxia Temporary loss of nerve conduction—often caused by pressure causing ischaemia • Axonotmesis Damage to the nerve fibre, but nerve tube is intact— the chance of successful nerve regrowth and a good recovery ishigh • Neurotmesis—Divided nerve—lack of guidance to the regrowing fibrils gives d chance of a good recovery, and a neuroma may develop Median nerve damage  The median nerve controls grasp. Damage causes inability to lift the thumb out of the plane of the palm (abductor pollicis brevis failure) and loss of sensation over the lateral side of thehand. Ulnar nerve damage Injury distal to the wrist causes a claw hand deformity, loss of abduction/adduction of the fingers, and sensory loss over the little finger and a variable area of the ring finger. Radial nerve damage  The radial nerve opens the fist—injury produces wrist drop and variable sensory loss including the dorsal aspect of the root of thethumb. Tendon injury  Can occur due to attrition or lacerations of the hand or wrist. Examine hand function. Always ensure no other structures are damaged before suturing skin wounds. Extensor or flexor tendons can be affected. Refer—primary surgical repair is usually the treatment of choice. Vascular injury Can occur due to trauma/lacerations of the hand or wrist. Check perfusion and temperature of fingers and examine pulses. Ensure no other structures are damaged before suturing skin wounds. Refer all vascular injuries for specialist assessment and management. Work-related upper limb pain Work-related pain in the arm ±

wrist, e.g. due to keyboard use. Overuse syndrome. Often termed repetitive strain injury (RSI). Diagnosis of exclusion—no physical signs. Exclude other conditions, e.g. carpal tunnel syndrome (CTS), tenniselbow. Management  Reassure—condition is curable, continue work, but avoid the aggravating activity, liaise with work to ensure evaluation of workstation ergonomics. Gradually reintroduce activity. Physiotherapy may help. Explore psychological and work-related issues. Amultidisciplinary approach is needed. 0 Work-related upper limb pain is a notifiable industrial disease. X Existence of RSI has been challenged—rigorous assessment often reveals undiagnosed causes ofpain.

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Complex regional pain disorder  (also known as reflex sympathetic

dystrophy or algodystrophy). Pain ± vasomotor changes in a limb l loss of function. Most common in the hand and wrist. Usually follows trauma— but the trauma may be trivial and signs may appear weeks/months later. Signs: pain at rest exacerbated by movement and light touch, swelling, discoloration, temperature changes, abnormal sensitivity, sweating, and loss of function. X-ray may show osteopenia. Management  Physiotherapy improves prognosis if started early; analgesia (NSAIDs, opioids, and/or nerve painkillers). Refer to pain clinic or rheumatology for specialist treatments, e.g. nerve block, spinal cord stimulation, CBT, and/or graded motor imagery.

Tenosynovitis Inflammation of the tendon sheath—often due to unaccustomed activity (e.g. gardening). May affect extensor or flexor tendons. Pain is often worse in the morning. Presents with swelling and tenderness over the tendon sheath and pain on using the tendon. Treat with rest and NSAIDs. If not settling, an injection of steroid into the tendon sheath may help. 0 Notifiable industrial disease if work-related. De Quervain’s tenosynovitis Tenosynovitis of thumb extensor and abductor tendon sheaths. Pain over radial styloid and on forced adduction/ flexion of the thumb. Treat with thumb splint ± local steroid injection. Refer if not settling. F.de Quervain (1868–1940)—Swiss surgeon.

Carpal tunnel syndrome Pain in the radial 3½ digits of the hand

±numbness, pins and needles, and thenar wasting. Due to compression of the median nerve as it passes under the flexor retinaculum. Worse at night. Symptoms are improved by shaking the wrist. Associations: pregnancy, hypothyroidism, DM, obesity, and carpal arthritis. Investigations  Phalen’s test—hyperflexion of wrist for 1min triggers symptoms; Tinel’s test—tapping over the carpal tunnel causes paraesthesiae; request nerve conduction studies if diagnosis is indoubt. Management  GP treatment—night splints may help ± carpal tunnel steroid injection (b p.166). Less likely to help if age >50y or symptoms >10mo. If GP treatment fails, constant paraesthesiae and/or triggering of fingers, refer to orthopaedics for division of the flexor retinaculum.

Kienböck’s disease  The lunate bone develops patchy necrosis after acute or chronic injury. The patient is usually a young adult complaining of aching and stiffness of one wrist. Examination: tenderness in the centre of the back of the wrist ± limitation of wrist extension. X-ray is normal at first but later shows i density of the lunate ± deformity. Refer for orthopaedic opinion. R. Kienböck (1871–1953)—Austrian radiologist. Osteoarthritis in thehand • Heberden’s nodes—swellings of DIP joints. No treatment needed • Bouchard’s nodes—swellings of PIP joints. No treatment needed First carpometacarpal OA  Pain and swelling at the base of the thumb. Thumb becomes stiff. Asplint or steroid injection can be helpful. If pain persists surgery (trapeziectomy) mayhelp.

Wrist and hand problems

Dupuytren’s contracture  Palmar fascia contracts so that the fingers (typically the right fifth finger) cannot extend. Prevalence: 10% 4 > 65y (more if family history). Less common in women. Associations: smoking; alcohol; heavy manual labour; trauma; DM; phenytoin; Peyronie’s disease; AIDS. Often simple reassurance suffices. Consider referral for surgery (fasciotomy or fasciectomy) if MCP joint contracture >30° or PIP/DIP joint contracture >10°. G. Dupuytren (1777–1835)—French surgeon. Trigger finger  Nodules on the tendon can occur spontaneously and in RA and DM. Most common in ring and middle fingers. The nodule can be palpated moving with the tendon. Pain and triggering (the finger is in fixed flexion and needs to be flicked straight by the other hand) occur because the nodule jams in the tendon sheath. Management: local steroid injection or refer for surgical release. Mallet finger The fingertip droops due to avulsion of the extensor tendon attachment to the terminal phalanx (see Figure 15.2). Refer for X-ray. Management: a plastic splint which holds the terminal phalanx in extension is worn for 6wk to help union (must not be removed). Arthrodesis may be needed if healing does notoccur. Gamekeeper’s thumb  Forced thumb abduction causes rupture of the ulnar collateral ligament. Can occur on wringing a pheasant’s neck—hence the name, or, more commonly, by catching the thumb in the matting on a dry ski slope. The thumb is very painful and pincer grip weak. Refer—open surgical repair is the most effective treatment. Nail injuries Avulsed nail  Protect the nail bed of an avulsed nail with soft paraffin and gauze, check tetanus status, and give antibiotic prophylaxis (e.g. flucloxacillin 500mg qds for 7d). Partially avulsed nails need removing under ring block to exclude an underlying nail bed injury—the nail is replaced to act as a splint to the nail matrix. Subungual haematoma  A blow to the finger can cause bleeding under the nail—very painful due to pressure build-up. Relieve by trephining a hole through the nail using a 19 gauge needle (no force required; just twist the needle as it rests vertically on the nail) or a heated point (e.g. of a paper clip or cautery instrument). Of benefit up to 2d after injury.

Polydactyly  Extra digits can vary from small fleshy tags to complete duplications. They may be an isolated defect or associated with syndromes. Small fleshy tags are removed in the first few months. For extra digits that are firmly fixed or involving tendons or joints, surgery is delayed until the child is >1y. Refer to orthopaedics or plastic surgery. Syndactyly Digits may be joined by a web of skin or more firmly fused. Webbing is usually mild and treatment is for cosmetic reasons if at all. Where digits are fused separation and skin grafting is carried out at 74y. Refer to plastic surgery.

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Hip and pelvis problems History  Pain on walking? Pain at rest? Hip joint pain is usually felt in the groin (see Table 15.4). Referred pain is often felt in the knee. Hip disease results in d walking distance, difficulty climbing stairs and getting out of lowchairs. Examination • Look Watch the patient walk—hip disease l limp or waddlinggait • Feel Joint tenderness is just distal to the midpoint of the inguinal ligament • Move Passive movement with the patient lying supine. Check range of movement—pain reproduced on movement? Crepitus? • Measure Hip disease is often associated with shortening of the affected leg—true leg length:anterior superior iliac spine l medial malleolus; apparent leg length:umbilicus l medial malleolus • Trendelenburg test Ask the patient to stand on one leg and lift the foot on the contralateral side off the ground. Place your fingers on the anterior superior iliac spines. If the pelvis sags on the unsupported side (+ve Trendelenburg sign) the hip on which the patient is standing is painful or has a weak/mechanically disadvantaged gluteus medius 0False +ve in10%.

Malignancy Hip and pelvis are common sites for s malignancy. Pain is severe and unremitting, day and night. Often accompanied by weight loss. X-ray may show no abnormalities or reveal lytic or sclerotic deposits. Bone scan is diagnostic but may miss myeloma. Depending on clinical circ*mstances either refer for specialist advice (oncologist, radiotherapist) or to palliative care. Treat with analgesia meanwhile. High risk of pathological fracture. Osteoarthritis of the hip Major cause of hip pain and disability. Incidence i with age; 4 8 5. Predisposing factors: past hip disease (e.g. Perthes’) or trauma; unequal leg length. Presentation  Pain may be diffuse and felt in hip region, thigh, or knee. Relieved by rest in early stages of disease. Signs: d internal rotation and abduction of hip, with pain at extremes of movement; antalgic gait; eventually fixed flexion of the hip. Investigation: X-ray may confirm diagnosis but is often not needed. There is poor correlation between X-ray changes and pain felt. Perform Oxford Hip Score (see Table15.5). Table15.4  Causes of pain around thehip Pain

Causes

Buttock pain

PMR, sacroiliitis, vascular insufficiency, referred from back

Groin pain

Hip joint disease (OA, RA, Paget’s, osteomalacia), fracture, osteitis pubis, hernia, psoas abscess

Lateral thigh pain

Trochanteric bursitis, referred pain from back, enthesitis (spondylarthropathies), gluteus medius tear, meralgia paraesthetica, fascia lata syndrome

Hip and pelvis problems

Table15.5  Oxford Hip Score 1. During the past 4 weeks . . . How would you describe the pain you usually have from your hip? None (4)

Very mild (3)

Mild (2)

Moderate (1)

Severe (0)

2. During the past 4 weeks . . . Have you had any trouble with washing and drying yourself (all over) because of your hip? No trouble at all (4)

Very little trouble (3)

Moderate trouble (2)

Extreme difficulty (1)

Impossible to do (0)

3. During the past 4 weeks . . . Have you had any trouble getting in and out of a car or using public transportation because of your hip? (whichever you tend to use) No trouble at all (4)

Very little trouble (3)

Moderate trouble (2)

Extreme difficulty (1)

Impossible to do (0)

4. During the past 4 weeks . . . Have you been able to put on a pair of socks, stockings or tights? Yes, easily (4)

With little difficulty (3)

With moderate With extreme No, impossible difficulty (2) difficulty (1) (0)

5. During the past 4 weeks . . . Could you do the household shopping on your own? Yes, easily (4)

With little difficulty (3)

With moderate With extreme No, impossible difficulty (2) difficulty (1) (0)

6. During the past 4 weeks . . . For how long have you been able to walk before pain from your hip becomes severe (with or without a stick)? No pain/ for 16–30 min (3) ≥30 min (4)

5–15 min (2)

Around the Not at all (0) house only (1)

7. During the past 4 weeks . . . Have you been able to climb a flight of stairs? Yes, easily (4)

With little difficulty (3)

With moderate With extreme No, impossible difficulty (2) difficulty (1) (0)

8. During the past 4 weeks . . . After a meal (sat at a table), how painful has it been for you to stand up from a chair because of your hip? Not at all painful (4)

Slightly painful (3)

Moderately painful (2)

Very painful (1) Unbearable (0)

9. During the past 4 weeks . . . Have you been limping when walking because of your hip? Rarely/never Sometimes or Often, not just (4) just at first (3) at first (2)

Most of the time (1)

All of the time (0)

10. During the past 4 weeks . . . Have you had any sudden, severe pain - ‘shooting’, ‘stabbing’ or ‘spasms’ - from the affected hip? No days (4) 1 or 2 days (3) Some days (2) Most days (1) Every day (0) 11. During the past 4 weeks . . . How much has pain from your hip interfered with your usual work (including housework)? Not at all (4) A little bit (3) Moderately (2)

Greatly (1)

Totally (0)

12. During the past 4 weeks . . . Have you been troubled by pain from your hip in bed at night? No nights (4) 1 or 2 nights (3) Some nights (2) Most nights (1) Every night (0) Reproduced with permission from Isis Innovation Limited, 1998.

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Management of hip osteoarthritis Analgesia (e.g. regular paracetamol, NSAIDs), education, weight d, exercise, correction of unequal leg length. Walking stick ± shock-absorbing shoe insoles can help. Consider referral for physiotherapy (muscle strengthening exercises may d pain) or, if significant impairment in functioning (e.g. Oxford Hip Score ≤20) to orthopaedics for hip resurfacing or replacement. Total hip replacement  >90% achieve good result. Most last >15y. Post-op care: risk of dislocation in the first 6wk—advise to avoid crossing legs; take care with transfers; use a walking stick; no driving for 6wk. Physiotherapy is usually arranged via secondarycare. Hip dislocation Occurs in front seat passengers in car accidents as the knee strikes the dashboard. Reduction under anaesthetic is required. Greater trochanter pain (trochanteric bursitis)  Can mimic ± coexist with hip OA. May be associated with muscle weakness around the hip. Diagnosis: point tenderness over the greater trochanter. Management  Consider local steroid injection if trochanteric bursitis is likely. Refer to physiotherapy for exercises to strengthen hip musculature to prevent recurrence. Meralgia paraesthetica  Burning/numbness in the upper lateral aspect of the thigh due to compression of the lateral cutaneous nerve of the thigh. Risk factors: pregnancy, obesity, DM. Examination: extension of the hip or deep palpation just below the anterior superior iliac spine provokes symptoms. Treatment: analgesia (including neuropathic painkillers), TENS ± local steroid injection. Rarely surgical decompression is needed. Fascia lata syndrome  Inflammation of the fascia lata causing pain in the lateral thigh. Often due to overuse or weak musculature around the hip. Treatment is with rest ± referral to physiotherapy. Hip infection  Presents with hip pain, d weight, night sweats, and rigors. Be aware of infection in patients with RA, hip prosthesis, or immunocompromise. Refer for investigation. X-rays are often unhelpful—bone scan is non-specific. Admit for USS-guided drainage, bed rest, and IV antibiotics. Avascular necrosis May present with hip pain. Have a high level of suspicion in patients with risk factors—SLE, sickle cell disease, high alcohol consumption, pregnancy, or corticosteroids. X-ray or bone scan may confirm diagnosis but MRI is most sensitive. Specialist management is needed. Usually progresses to causeOA. Pubic symphysis dehiscence Painful condition occuring in late pregnancy that may persist after delivery. The pubic symphysis separates resulting in low abdominal pain which may be accompanied by low back pain and radiate down both thighs. Pain is constant and worse on movement. It resolves on rest. Examination reveals a soft abdomen and obstetric examination is normal. Advise simple analgesia (paracetamol 1g qds). Rest in a semi-recumbent position when in pain. Refer for physiotherapy, especially if still a problem in the puerperium. Most resolve spontaneously within several months of delivery. Some persist and need specialist referral.

Hip and pelvis problems

The limpingchild •  If a child is limping, take it seriously. Look for a problem • Children find it difficult to localize pain. Pain can be referred from the hip to the knee. Examine the whole limb carefully • Other causes of referred pain include:spinal pathology, psoas spasm from GI pathology (e.g. appendicitis) • Limping without pain is uncommon and may be due to undiagnosed developmental dysplasia of the hip—b p.854 Transient synovitis of the hip (irritable hip)  The most common reason for limping in childhood. Peak age: 2–10y. 4 > 5. The child is usually well but complains of pain in the hip or knee and may refuse to weight-bear. Often occurs after a viral infection. Cause is unknown. Exclude septic arthritis—refer to orthopaedics. Usually resolves in 7–10d without treatment. Perthes’ disease  Pain in the hip or knee, limp, and limited hip movement developing over 71mo. Due to avascular necrosis of the femoral head. Bilateral in 10%. Peak age: 4–7y (range 3–11y). 4 :584:1. Management If suspected refer for X-ray and to orthopaedics. Treatment is with rest, X-ray surveillance, bracing, and/or surgery depending on severity. Usually heals over 2–3y. Joint damage may cause early arthritis. Risk factors for poor outcome include: • 5 • Onset>8y • Involvement of the whole femoralhead • Pronounced metaphyseal rarefaction • Lateral displacement of the femoralhead G.C. Perthes (1869–1927)—German surgeon.

Slipped upper femoral epiphysis The upper femoral epiphy-

sis slips with respect to the femur, usually in a postero-inferior direction. Bilateral in 20%. Incidence: 1:100,000. Peak age: 10–15y. 4:58 3:1. Typically affects obese, underdeveloped children or tall, thinboys. Presentation  Pain at rest in the groin, hip, thigh, or referred to the knee; limp and/or pain on movement; d hip movements—particularly abduction and medial rotation. The affected leg may be externally rotated and shortened. Management Confirm diagnosis on X-ray (include lateral views)— shows backwards and downwards slippage of the epiphysis. Refer to orthopaedics—surgical pinning or reconstructive surgery is needed. Monitoring of the other hip is essential. Complications include:avascular necrosis; coxa vara; early OA; slipped epiphysis on the contralateralside. Developmental dysplasia of the hip  bp.854

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Knee problems History • Trauma History of injury—ask about degree and direction offorce • Pain/stiffness Attempt to distinguish well-localized mechanical pain and diffuse inflammatory/degenerativepain • Deformity Swelling? If injury, time of onset of swelling in relation to history (immediate effusion suggests haemarthrosis; post-traumatic effusions appear later). Knock-knees or bow-legs? • Function Do the Oxford Knee Score (see Table15.6)

Examination  Always compare the twoknees. • Look Watch the patient walk. Look at the knees whilst standing— varus/valgus deformity? Ask the patient to lie down. Note quadriceps wasting, scars, skin changes, swelling, and deformity. Aspace under the knee viewed laterally suggests a fixed flexion deformity. With legs extended, lift both feet off the bed to demonstrate hyperextension • Feel Feel the quadriceps for wasting and palpate the knee for warmth. Check the joint line, collateral ligaments, tibial tubercle, and femoral epicondyles for tenderness. Palpate the popliteal fossa for a Baker’s cyst. Check for an effusion. Test for patellofemoral lesions by sliding the patella sideways across the underlying femoral condyles • Move With the patient lying on his back check active and passive range of movement—pain reproduced on movement? Crepitus? Test the medial and lateral collateral ligaments and cruciate ligaments • Measure Quadriceps diameter 18cm up from the joint line in adults 0 Knee pain can be referred from the hip so examine the hip aswell. Osteoarthritis of the knee  Very common; X-ray evidence of OA is even more common. Treatment: education; glucosamine; analgesia (paracetamol ± NSAIDs); exercise (refer to physiotherapy). Suggest using a walking stick. Steroid injection can be helpful in some patients. If pain and disability are severe (e.g. Oxford Knee Score ≤16), refer to orthopaedics for consideration of total or partial knee replacement. Knee replacement is a very successful procedure resulting in d pain and i mobility. 95% prostheses last>10y. Infection of the knee joint  Most commonly infected joint. Signs: hot, red, swollen, painful knee. Differential diagnosis: Reiter’s disease, gout, pseudogout, traumatic effusion, RA. If infection is suspected refer as an emergency to rheumatology or orthopaedics for investigation. 0 Do not give antibiotics until the joint has been aspirated. Non-traumatic knee effusion  Common causes:gout, RA, calcium pyrophosphate dehydrate disease (pseudogout), spondylarthropathies (including reactive arthritis). Consider FBC, ESR, rheumatoid factor, anti-nuclear antibody, LFTs, bone biochemistry, and thyroid function tests. Drain effusion (or refer to rheumatology to drain) and send fluid for polarized light miscroscopy (for crystals) and microbiology (?infection). Management  If no infection, inject with long-acting steroid (b p.164). If recurrent and no cause found, refer to rheumatology.

Knee problems

Table15.6  Oxford Knee Score 1. During the past 4 weeks . . . How would you describe the pain you usually have from your knee? None (4)

Very mild (3)

Mild (2)

Moderate (1)

Severe (0)

2. During the past 4 weeks . . . Have you had any trouble with washing and drying yourself (all over) because of your knee? No trouble at all (4)

Very little trouble (3)

Moderate trouble (2)

Extreme difficulty (1)

Impossible to do (0)

3. During the past 4 weeks . . . Have you had any trouble getting in and out of a car or using public transport because of your knee? (whichever you tend to use) No trouble at all (4)

Very little trouble (3)

Moderate trouble (2)

Extreme difficulty (1)

Impossible to do (0)

4. During the past 4 weeks . . . For how long have you been able to walk before pain in your knee becomes severe (with or without a stick)? No pain/ for 16–30 min (3) ≥60 min (4)

5–15 min (2)

Around the Not at all (0) house only (1)

5. During the past 4 weeks . . . After a meal (sat at a table), how painful has it been for you to stand up from a chair because of your knee? Not at all painful (4)

Slightly painful (3)

Moderately painful (2)

Very painful Unbearable (0) (1)

6. During the past 4 weeks . . . Have you been limping when walking, because of your knee? Rarely/never Sometimes or Often, not just Most of the (4) just at first (3) at first (2) time (1)

All of the time (0)

7. During the past 4 weeks . . . Could you kneel down and get up again afterwards? Yes, easily (4)

With little With moderate With extreme No, impossible difficulty (3) difficulty (2) difficulty (1) (0)

8. During the past 4 weeks . . . Have you been troubled by pain from your knee in bed at night? No nights (4) 1 or 2 nights (3) Some nights (2) Most nights (1) Every night (0) 9. During the past 4 weeks . . . How much has pain from your knee interfered with your usual work (including housework)? Not at all (4) A little bit (3) Moderately (2)

Greatly (1)

Totally (0)

10. During the past 4 weeks . . . Have you felt that your knee might suddenly ‘give way’ or let you down? Rarely/never Sometimes or Often, not just Most of the (4) just at first (3) at first (2) time (1)

All of the time (0)

11. During the past 4 weeks . . . Could you do the household shopping on your own? Yes, easily (4)

With little With moderate With extreme No, impossible difficulty (3) difficulty (2) difficulty (1) (0)

12. During the past 4 weeks . . . Could you walk down one flight of stairs? Yes, easily (4)

With little With moderate With extreme No, impossible difficulty (3) difficulty (2) difficulty (1) (0)

Reproduced with permission from Isis Innovation Limited, 1998.

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Bipartite patella  Detected on X-ray. Usually asymptomatic incidental finding but can cause pain due to excessive mobility of a patella fragment. If troublesome refer for fragment excision. Patellar dislocation  Lateral dislocation of the patella and tearing of the medial capsule/quadriceps can occur due to trauma. More common in young people and if joint hypermobility syndrome. Patient is in pain and unable to flex knee. Refer via A&E or orthopaedics for reduction. Recurrent subluxation of the patella Medial knee pain + knee ‘gives way’ due to lateral subluxation of the patella. Most common in girls with valgus knees. Associations: familial, hypermobility, high-riding patella. Signs:i lateral patella movement and +ve apprehension test (pain and reflex contraction of quadriceps on lateral patella pressure). Refer to physiotherapy for vastus medialis exercises. If that is unhelpful, refer to rheumatology to exclude a hereditary connective tissue disorder and/or to orthopaedics for consideration of lateral retinacular release. Patella tendinitis  Small tear in the patella tendon causes pain. Most commonly seen in athletes. Differential includes inferior patellar pole enthesitis (spondylarthropathies), fat-pad syndrome, anterior cartilage lesion, and bursitis. Diagnosis is with USS. Treatment is with rest, NSAIDs ± steroid injection around (not into) the tendon. Bursitis Prepatellar bursitis (housemaid’s knee) is associated with excess kneeling. Vicar’s knee (infrapatellar bursitis) is associated with upright kneeling. Avoid aggravating activity, aspirate ± steroid injection (drecurrence). If infected treat with antibiotics ± refer for drainage. Baker’s cyst Popliteal cyst (herniation of joint synovium) can cause swelling and discomfort behind the knee. Usually caused by a degenerative knee. Rupture may result in pain and swelling in the calf mimicking DVT. Treat underlying knee synovitis. Surgical cyst removal may be necessary if persistent problems. W.M. Baker (1839–96)—English surgeon. Collateral ligament injury Common in contact sports. Causes knee effusion if severe ± tenderness over the injured ligament. Collateral ligaments provide lateral stability to the knee. Normally there is 5° the ligament may be ruptured. Treat with rest, knee support, analgesia. Refer to orthopaedics if rupture is suspected. Cruciate ligament injury Cruciate ligaments provide anterior/ posterior knee stability. Assessment can be difficult. • Anterior cruciate tears Result from a blow to the back of tibia ± rotation when the foot is fixed on the ground. Signs: effusion and +ve drawer test (supine with foot fixed and knee at 90°, pull the tibia forward—test is +ve if the tibia moves forward on the femur) • Posterior cruciate tears Caused, e.g. when the knee hits the dashboard in car accidents. Reverse drawer test is +ve (supine with knee at 90°; apply pressure to push the tibia backwards—test is +ve if the tibia moves backward on the femur) Management  Refer to orthopaedics if suspected. Splinting and then physiotherapy helps most (60%) but some require reconstructive surgery— consider urgent referral if keen sportsman.

Knee problems

Loose bodies in the knee  May result in locking of the joint and/or effusion. Causes: OA, chip fractures, osteochondritis dissecans, synovial chondromatosis. If problematic refer for removal. Osteochondritis dissecans Necrosis of articular cartilage and underlying bone. Can cause loose body formation. Cause unknown. Seen in young adults l pain after exercise and intermittent knee swelling ± locking. Predisposes to arthritis. Refer for expert management. Meniscal lesions Twisting with the knee flexed can cause medial (bucket handle) meniscal tears and adduction with internal rotation can cause lateral cartilage tears. Symptoms/signs: • Locking of the knee—extension is limited due to cartilage fragment lodging between the condyles • Giving way of the knee        • Tender jointline • +ve McMurray’s test—rotation of the tibia on the femur with flexed knee followed by knee extension causes pain and a click, as the trapped cartilage fragment is released. X Reliability of this test is debated Management  Refer for MRI ± arthroscopy. Treated by removal of the torn meniscal fragment. Meniscal cyst  Pain + swelling over the joint line due to a meniscal tear. Lateral cysts are more common than medial. The knee may click and give way. Refer for arthroscopy—removal of damaged meniscus relievespain. Chondromalacia patellae  Common in teenage girls. Pain

on walking up or down stairs or on prolonged sitting. Signs: pain on stressing the undersurface of the patella. Arthroscopy (indicated only in severe cases) reveals degenerative cartilage on the posterior surface of the patella. Treat with analgesia + physiotherapy (vastus medialis strengthening d pain in 80%). If persistent, exclude spondylarthropathy ( b p.518) and refer to orthopaedics for arthroscopy. Osgood–Schlatter disease Seen in athletic teenagers. Pain and tenderness ± swelling over the tibial tubercle. X-rays not required. Avoid aggravating activities. Usually settles over a few months. If not settling refer to orthopaedics or rheumatology for further assessment. R.B. Osgood (1873–1956)—US orthopaedic surgeon; C.B. Schlatter (1864–1934)—Swiss physician.

Bow-legs and knock-knees in children  • Genu varum (bow-legs) Outward curving of the tibia usually associated with internal tibial torsion. Except in severe cases always resolves spontaneously. Severe cases raise the possibility of rickets or other rare developmental disorders—refer for orthopaedic opinion. • Genu valgum (knock-knees) Common amongst 2–4y olds. Innocent if symmetrical and independent of any other abnormality. Severe, progressive cases suggest rickets—refer forX-ray.

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Ankle and foot problems History Trauma; i activity, e.g. walking or running a long way for the patient; feeling of instability; pain/stiffness (relation to weight-bearing; localized/diffuse); deformity (problems getting shoes, shoes wear in odd places, or shoes are always uncomfortable); interference with activities. Examination  Compare one foot with theother: • Look Watch the patient walk normally and on tiptoe. Look at the foot with the patient seated. Check for deformities, the colour of the foot, and any skin/nail changes. Check the shoes for any abnormal patterns of wear (wear is normally under the ball of the foot medially and posterolaterally at theheel) • Feel Is there any tenderness? Palpate any swellings. Check pulses and skin temperature • Move Assess active and passive movements of the ankle, subtalar, mid-tarsal, and toe joints systematically. Check range of movement of joints andpain • Neurology Check sensation if patient reports any loss of sensation Ankle, foot, and toe fracture  b p.1110 Achilles tendonitis Inflammation of the Achilles tendon may be related to overuse or a spondylarthropathy. Presents as a painful local swelling of the tendon. Advise rest. NSAIDs, heel padding, physiotherapy ± steroid injection may help (never inject into the tendon). If persistent refer to rheumatology. Ruptured Achilles tendon  Presents with a sudden pain in the back of the ankle during activity (felt as a ‘kick’). The patient walks with a limp. There is some plantar flexion, but the patient cannot raise the affected heel from the floor when standing on tiptoe. A‘gap’ can usually be felt in the tendon. Calf squeeze test is–ve (squeezing the calf muscles results in movement of the foot if the Achilles tendon is intact). Refer immediately for consideration of repair. The alternative is immobilization in a splint with the foot plantar flexed. Pes cavus High foot arches may be idiopathic, due to polio, spina bifida, or other neurological conditions. Toes may claw. Padding under the metatarsal heads relieves pressure. Operative treatment—soft tissue release or arthrodesis—straightens toes. Can lead to tarsal bone OA causing pain—refer for fusion. Foot drop Patients trip frequently or walk with a high stepping gait.

On examination, patients are unable to walk on their heels and cannot dorsiflex their foot. Check ankle jerk. Causes: • Common peroneal palsy, e.g. due to trauma—normal anklejerk • Sciatica—ankle jerkabsent • L4, L5 root lesion—ankle jerk may beabsent • Peripheral motor neuropathy, e.g. alcoholic—ankle jerk weak orabsent • Distal myopathy—ankle jerk weak orabsent • Motor neurone disease—i anklejerk

Ankle and foot problems

Club foot (talipes) Consists of inversion of the foot, adduction of forefoot relative to hindfoot, and equinus (plantar flexion). Positional talipes  Moulding deformity seen in neonates. The foot can be passively everted and dorsiflexed to the normal position. Treatment is with physiotherapy. Follow-up to check the deformity is resolving. True talipes The foot cannot be passively everted and dorsiflexed to the normal position. Refer to orthopaedics. Treatment is with physiotherapy, splints ± surgery. Flat feet (pes planus) Low medial arch. All babies and toddlers have flat feet. The arch develops after 2–3y of walking. Persistent flat feet may be familial or due to joint laxity. If pain-free, foot is mobile, and the patient develops an arch on standing on tiptoe (‘flexible’ foot), no action is required. If painful may be helped by analgesia, exercises, or insoles. For severe pain, hind foot fusion is an option. Refer if the arch does not restore on tiptoeing (‘rigid’). In-toe and out-toegait  • In-toe Originates in the femur (persistent anteversion of the femoral neck), tibia (tibial torsion), or foot (metatarsus varus). Does not cause pain or affect mobility. Usually resolves by age5–6y • Out-toe Common 4 Presents with pain in the foot on walking. The head of the metatarsal is palpable and tender X-ray shows a wide, flat metatarsal

Treatment is usually conservative with cushioning of shoes and simple analgesia. If severe refer to orthopaedics. Excision of the metatarsal head may relieve pain

Freiberg’s Second disease and third metatarsal heads

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Tender heel pad  Dull throbbing pain under the heel. Develops a few

months after heel trauma. May be due to plantar fasciitis, bursitis, or tendinitis. Treat with rest and heel padding. Refer to physiotherapy—ultrasound treatment can help. Blind steroid injections into the fat pad are not recommended. In persistent cases refer to rheumatology.

Plantar fasciitis/bursitis Common cause of inferior heel pain, especially amongst runners. Pain is worst when taking the first few steps after getting out of bed. Usually unilateral and generally settles in 80% of tumours: Osteosarcoma and the Ewing’s family of tumours Present with aching bone pain, swelling ± pathological fracture. If X-ray is normal, but symptoms persist, consider checking bone function tests, re-X-raying, discussing the patient with a specialist or referral. Treatment involves surgery and chemotherapy. Overall 5y survival is 50–80%. Adult soft tissue sarcoma of limb or trunk  Usually presents with a palpable lump. The most common tumours are leiomyosarcoma, liposarcoma, and synovial sarcoma. Treated with surgery ± radiotherapy (high-grade tumours). Chemotherapy is reserved for palliation. Kaposi’s sarcoma  bp.746 Intra-abdominal sarcoma Usually presents late. Often arises in the retroperitoneum. If possible, surgery is the main treatment. Local relapse is common and often not responsive to cytotoxic therapy. Rhabdomyosarcoma  Originates from striated muscle. Presents usually in children 60% long-term survival).

Further information NICE Referral guidelines for suspected cancer (2005) M www.nice.org.uk

Patient information and support Brittle Bone Society F 01382 204446 M www.brittlebone.org Paget’s Association F 0161 799 4646 M www.paget.org.uk Sarcoma UK F 020 7250 8271 M www.sarcoma.org.uk

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Rickets and osteomalacia Vitamin D deficiency causes rickets in children and osteomalacia in adults. The body needs 710 micrograms of vitamin D per day to maintain healthy bones. The body makes its own vitamin D when sunlight falls on the skin in the summer months but a diet with adequate vitamin D is needed to maintain the supply in the winter—especially for people who do not get out or for cultural or religious reasons are completely shielded from the sun by their clothing. For dietary sources of vitamin D and calcium see Tables 15.8 and 15.9.

Clinical features of rickets • Bone pain/tenderness:arms, legs, spine,pelvis • Skeletal deformity:bow-legs, pigeon chest (forward projection of the sternum), rachitic rosary (enlarged ends of ribs), asymmetrical/ odd-shaped skull due to soft skull bones, spinal deformity (kyphosis, scoliosis), pelvic deformities • Pathological fracture • Dental deformities—delayed formation of teeth, holes in enamel, i cavities • Muscular problems—progressive weakness, d muscle tone, musclecramps • Impaired growth l short stature (can be permanent)

Clinical features of osteomalacia • Bone pain—diffuse, particularly inhips • Muscle weakness • Pathological fractures • Low calcium l perioral numbness, numbness of extremities, hand and feet spasms, and/or arrhythmias

Causes and management Dietary deficiency (80y. Secondary rickets/osteomalacia  Due to other disease, e.g. malabsorption, liver disease, renal tubular disorders, or chronic renal failure. Treat underlying cause/supplement Ca2+ and vitaminD. Vitamin D-dependent rickets  Rare autosomal recessive inherited disorder resulting in an enzyme deficit in the metabolism of vitamin D.Refer for specialist care. Treated with vitamin D and Ca2+ supplements. Hypophosphataemic rickets (vitamin D-resistant rickets)  X-linked dominant trait resulting in d proximal renal tubular resorption of phosphate. Parathyroid hormone and vitamin D levels are normal. Specialist management is needed. Treatment is with phosphate replacement ± calcitriol.

Rickets and osteomalacia

Table15.8  Approximate vitamin D content of commonfoods* Food

Serving

Vitamin D (micrograms)

Margarine

10g (1/2oz)

0.8

Eggs

1 size 3

1.1

Cheese

60g (2oz)

0.2

Milk

0.15L (1/4 pint)

0.05

Butter

10g (1/2oz)

0.1

Fortified cereals

30g (1oz)

0.5

Herring

100g (31/2oz)

16.5

Mackerel

100g (31/2oz)

8

Sardines

100g (31/2oz)

7.5

Tinned tuna

100g (31/2oz)

4

Tinned salmon

100g (31/2oz)

12.5

Kipper

100g (31/2oz)

13.5

* Recommended daily intakes:birth to 50y—5 micrograms; 50 to 70y—10 micrograms; >70y—15 micrograms

Table15.9  Approximate calcium content of common foods** Food

Serving

Calcium (mg)

Whole milk

0.2L (1/3 pint)

220

Semi-skimmed milk

0.2L (1/3 pint)

230

Hard cheese

30g (1oz)

190

Cottage cheese

115g (4oz)

80

Low-fat yoghurt

150g (5oz)

225

Sardines (including bones)

60g (2oz)

310

Brown or white bread

3 large slices

100

Wholemeal bread

3 large slices

55

Baked beans

115g (4oz)

60

Boiled cabbage

115g (4oz)

40

Recommended daily intakes: birth to 6mo—210mg; 7mo to 1y—270mg; 1 to 3y—500mg; 4 to 8y—800mg; 9 to 18y—1300mg; 19 to 50y—1,000mg; >50y—1,200mg. **

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Osteoporosis Lifetime risk of osteoporotic fracture is 1:3 in 5 and 1:5 in 4 (>200,000 fractures/y in the UK). The main morbidity and financial costs of osteoporosis relate to hip fracture where incidence i steeply >70y. Treatment aims to prevent fracture.

Definitions • T-scores compare bone mineral density (BMD) of the subject with the young adult mean (age30y) • Osteoporosis is defined as BMD >2.5 standard deviations (SD) below the young adult mean (T-score of–2.5). There is i relative risk of fracture x2–3 for each SD d inBMD • Osteopenia is diagnosed if T-score is between–1 and–2.5 • Z-scores compare BMDs of subjects and age-matched normal controls, i.e. they measure whether BMD is normal for the patient’s age. They cannot be used to diagnose osteoporosis or osteopenia, but may be useful in young patients to predict osteoporosis risk for the future

Causes  Osteoporosis may be 1o or 2o to other medical conditions: • Endocrine Hypogonadism (e.g. premature menopause, anorexia, androgen blockade, taking aromatase inhibitors), hyperthyroidism, hyperparathyroidism, hyperprolactinaemia, Cushing’s disease, type1DM • GI Coeliac disease or other causes of malabsorption, inflammatory bowel disease, chronic liver disease, chronic pancreatitis • Rheumatological RA, other inflammatory arthropathies • Other Immobility, multiple myeloma, haemoglobinopathy, systemic mastocytosis, CF, COPD, CKD, hom*ocystinuria Fragility fracture  Fracture sustained falling from ≤ standing height— includes vertebral collapse (may not be as a result of a fall). Previous fracture is a risk for future fracture. Common fractures: • Hip Associated with i mortality • Wrist Colles’ fracture • Osteoporotic vertebral collapse causes pain, d height, and kyphosis. Pain can take 3–6mo to settle and requires strong analgesia. Calcitonin is useful for pain relief for 3mo after vertebral fracture if other analgesics are ineffective Predicting fracture riskN  2 validated fracture risk prediction tools are available:FRAX (available from M www.shef.ac.uk/FRAX) and Qfracture (available from M www.qfracture.org). Qfracture does not require BMD measurement; FRAX can be performed without BMD measurement. Both provide information on 10y probability of hip or other osteoporotic fracture. Case-finding and further actions—see Figure15.3. Bone mineral density (BMD) measurement X-rays cannot be

used to measure BMD but are useful if vertebral fracture or metastases are suspected. Hip and lumbar spine BMD is measured using dual-energy X-ray absorptiometry (DEXA). Do not request without prior use of a risk prediction tool, e.g. FRAX (without BMD measurement) or Qfracture.

Osteoporosis

Glucocorticoid use Steroid use is a risk factor for osteoporosis.

Minimize steroid dose. For patients taking oral/high-dose inhaled steroids for >3mo or frequent courses of steroids, in addition: • Add bone protection agent (e.g. bisphosphonate) for patients >65y or with history of fragility fracture,or • Refer patients 80y, housebound, or institutionalized Regular exercise  Weight-bearing activity >30min/d d fracturerate Stop smoking  Women that stop smoking pre-menopause have a 25% d fracture rate post-menopause d alcohol consumption  To 5y l oversuppression of bone turnover and i bone fragility. Acute sub-trochanteric or mid-shaft femoral fractures are most common. XTo prevent this, a ‘drug holiday’ of 1–5y has been proposed for low-risk patients after 5y use—follow local guidance. Strontium ranelate  No longer recommended for p or s prevention of osteoporosis due to concerns regarding skin/hypersensitivity reactions and i risk of venous thromboembolism and CVD. May still be of benefit for those at high risk of fracture, who have no history of CVD and are intolerant of other medication. Patients taking strontium ranelate should have regular screening/monitoring to exclude CVD. Raloxifene  60mg od. Selective oestrogen receptor modulator (SERM). • For patients with previous fragility fracture if bisphosphonates are contraindicated/not tolerated or there is an unsatisfactory response with bisphosphonates (further fracture and/or d in BMD after ≥1y treatment). SERMs are not recommended for primary prevention of osteoporotic fractureN • Avoid if past history of DVT/PE, cholestasis, endometrial cancer, or undiagnosed vagin*l bleeding

Treatment options for osteoporosis

Denosumab  60mg sc every 6mo. Monoclonal antibody that d osteoclast activation and d bone resorption. • For post-menopausal osteoporosis when bisphosphonates are contraindicated/not tolerated and severe osteoporosisN • Can be used for women with severe CKD; correct hypocalcaemia before starting treatment. May cause osteonecrosis of thejaw HRT  (b p.712) Postpones post-menopausal bone loss and d fracturesC. Optimum duration of use is uncertain (>5–7y) but benefit disappears 51y HRT should not be considered first-line therapy for long-term prevention of osteoporosis. HRT remains an option where other therapies are contraindicated, cannot be tolerated, or if there is a lack of response; risks and benefits should be carefully assessed

Teriparatide Third-line for postmenopausal women and second-line for men with past history of fragility fracture if other treatments are not tolerated/ineffective and specific T-score and clinical criteria are met. Given by daily injection. Maximum duration of use is 18mo. Consider referral for consultant initiation if other treatment options are exhausted. Osteoporosis in men  Currently only bisphosphonates and teriparatide are recommended for treatment of osteoporosis inmen. Monitoring There is no consensus about duration of treatment for osteoporosis or monitoring of BMD during treatment. Circ*mstances in which repeat DEXA scanning might be necessary include: • Fragility fracture on treatment • If considering a change in treatment • When considering restarting therapy after a drug holiday Referral  Consider referral to an appropriate specialistif: • Another cause for fragility fracture is suspected (e.g. metastasis)—U • Fragility fracture on treatment—R • Unusual presentation of osteoporosis, e.g. pre-menopausal woman—R • For consideration of treatment with IV bisphosphonate, denosumab, or teriparatide—R U = urgent referral; R = routine referral. Osteopenia  If T-score of between–1 and–2.5, provide lifestyle advice. Repeat DEXA scan in72y. Further information NICE M www.nice.org.uk • Osteoporotic fractures—denosumab (2010) • Alendronic acid, etidronate, risedronate, raloxifene, strontium ranelate and teriparatide for the primary and secondary prevention of osteoporotic fragility fractures in postmenopausal women (2011) CSM Guidance Further advice on safety of HRT (12/2003) M www.mca.gov.uk

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Osteoarthritis Osteoarthritis (OA) is the most important cause of locomotor disability. It used to be considered ‘wear and tear’ of the bone/cartilage of synovial joints but is now recognized as a metabolically active process involving the whole joint—i.e. cartilage, bone, synovium, capsule, and muscle. The main reason for patients seeking medical help is pain. Level of pain and disability are greatly influenced by the patient’s personality, anxiety, depression, and activity, and often do not correlate well with clinicalsigns.

Risk factors  i age (uncommon 4;i in black and Asian

populations; genetic predisposition; obesity; abnormal mechanical loading of joint, e.g. instability; poor muscle function; post-meniscectomy; certain occupations, e.g. farming.

Symptoms and signs Joint pain ± stiffness, synovial thickening,

deformity, effusion, crepitus, muscle weakness and wasting, and dfunction. Most commonly affects hip, knee, and base of thumb. Typically exacerbations occur that may last weeks to months. Nodal OA, with swelling of the distal interphalangeal joints (Heberden’s nodes), has a familial tendency.

Investigations X-rays may show d joint space, cysts, and sclerosis in subchondral bone, and osteophytes. OA is common and may be a coincidental finding. Exclude other causes of pain, e.g. check FBC and ESR if inflammatory arthritis is suspected (normal or mildly i in OA—ESR >30mm/h suggests RA or psoriatic arthritis). Management of osteoarthritis in primary care  Employ a holistic approach. Assess effect of OA on the patient’s functioning, quality of life, occupation, mood, relationships, and leisure activities. Formulate a management plan with the patient that includes self-management strategies, effects of co-morbidities, and regular review.

Information and advice Give information and advice on all relevant aspects of osteoarthritis and its management. Arthritis Research UK produces a range of leaflets for patients. Use the whole multidisciplinary team, e.g. referto: • Physiotherapist for advice on exercises, strapping, and splints • OT foraids • Chiropodist for foot care and insoles • Social worker for advice on disability benefits and housing • Orthopaedics for surgery if significant disability/nightpain d load on the joint Weight reduction can d symptoms and may dprogression in knee OA. Using a walking stick in the opposite hand to the affected hip and cushioned insoles/shoes (e.g. trainers) can alsohelp. Exercise and improving muscle strength  d pain and disability, e.g. walking (for OA knee), swimming (for OA back and hip but may make neck worse), cycling (for OA hip or knee but may worsen patellofemoral OA). Refer to physiotherapy for advice on exercises especially isometric exercises for the less mobile.

Osteoarthritis

Pain control • Use non-pharmacological methods first (activity, exercise, weight d, footwear modification, walking stick,TENS, local heat/cold treatments) • Regular paracetamol (1g qds) is first-line drug treatment for all OA and/or topical NSAIDs for knee/hand OA only. Topical NSAIDs have less side effects than oral NSAIDs and are more acceptable to patients • Use opioids, oral NSAIDs, or COX2 inhibitors as second-line agents in addition to, or instead of paracetamol. Use the lowest effective dose for the shortest possible time. Co-prescribe a proton pump inhibitor (e.g. omeprazole 20mg od) with NSAIDs • Low-dose antidepressants, e.g. amitriptyline 10–75mg nocte (unlicensed), are a useful adjunct especially for pain causing sleep disturbance • Capsaicin cream can also be helpful for knee/handOAN Aspiration of joint effusions and joint injections  Can help in exacerbations. Some patients respond well to long-acting steroid injections—it may be worth considering a trial of a single treatment. Hyaluronic acid knee injections are not recommended byNICE. Complementary therapies 760% of sufferers from OA are thought to use CAM, e.g. copper bracelets, acupuncture, food supplements, dietary manipulation. There is good evidence chiropractic/osteopathy can be helpful for back pain, but otherwise evidence of effectiveness is scanty. Advise patients to find a reputable practitioner with accredited training who is a member of a recognized professional body and carries professional indemnity insurance. X Other drugs/supplements • Glucosamine It is controversial whether glucosamine modifies OA progression. It is available OTC but not recommended byNICE • Strontium ranelate d progression of OA, d pain, and i mobilityR. Place in OA management is yet to be determined Psychological factors have a major impact on the disability from OA. Education about the disease, and emphasis that it is not progressive in most people, is important. Seek and treat depression and anxiety with screening tools—b p.199

Refer • To rheumatology To confirm diagnosis if coexistent psoriasis (psoriatic arthritis mimics OA and can be missed by radiologists); rule out s causes of OA (e.g. pseudogout, haemochromatosis) if young OA or odd distribution; if joint injection is thought worthwhile but you lack expertise or confidence todoit • To orthopaedics If symptoms are severe for joint replacement. Refer as an emergency if you suspect joint sepsis

Further information NICE Osteoarthritis:the care and management of osteoarthritis in adults (2008) M www.nice.org.uk

Information and support for patients Arthritis Research UK F 0300 790 0400 M www.arthritisresearchuk.org Arthritis Care F 0808 800 4050 M www.arthritiscare.org.uk

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Rheumatoid arthritis Rheumatoid arthritis (RA) is the most common disorder of connective tissue affecting 71% of the UK population. It is an immunological disease, triggered by environmental factors, in patients with genetic predisposition. Disease course is variable with exacerbations and remissions. • Refer all suspected cases of rheumatoid arthritis to rheumatology— early treatment with disease-modifying drugs can significantly alter disease progression. Refer urgentlyNif: • Small joints of the hands/feet are affected • >1 joint is affected • There has been a delay of ≥3mo between onset of symptoms and seeking medicaladvice

Presentation

• Can present at any age—most common in middle age. 5:4 83:1 • Variable onset—often gradual but may beacute • Usually starts with symmetrical small joint involvement—i.e. pain, stiffness, swelling, and functional loss (especially in the hands); joint damage and deformity occurlater • Irreversible damage occurs early if untreated and can l deformity and joint instability • Other presentations—monoarthritis, migratory (palindromic) arthritis; PMR-like illness; systemic illness of malaise, pain, and stiffness Symptoms and signs  Predominantly peripheral joints are affected— symmetrical joint pain, effusions, soft tissue swelling, early morning stiffness. Progression to joint destruction and deformity. Tendons may rupture. Specific features—see Table15.10. Differential diagnosis  Diagnosis may not be easy—consider: • Bilateral carpal tunnel syndrome • Psoriatic arthritis • Other connective tissue disorders • NodalOA • Polymyalgia rheumatica if>50y • SLE (especially in 510mg/L). Boutonnière deformity

Swan neck deformity

Figure15.4  Boutonnière and swan neck deformities of the fingers

Information and support for patients Arthritis Research UK F 0300 790 0400 M www.arthritisresearchuk.org Arthritis Care F 0808 800 4050 M www.arthritiscare.org.uk

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Medication NSAIDs and simple analgesics (e.g. regular paracetamol). Provide symptomatic relief but do not alter the course of disease. Patients’ response to NSAIDs is individual—start with the least gastric-toxic, e.g. ibuprofen 200–400mg tds and alter as necessary, e.g. to naproxen 500mg bd. If the patient has a history of indigestion/gastric problems consider adding gastric protection, e.g. PPI, or, if there is no history of CVD, using a COX2 inhibitor, e.g. celecoxib 100mgbd. Corticosteroids Intra-articular injections of steroids (e.g. triamcinolone) can settle localized flares (e.g. knee or shoulder) and can be used up to 3x/y in any particular joint. Depot IM injections or IV infusions (pulses) can also help to settle an acute flare but offer short-term benefits with the risk of systemic side effects. Daily low-dose oral steroids help symptoms and there is some evidence that they can modify disease progression, but concerns about adverse side effects have limiteduse. Disease-modifying drugs (DMARDs) • Hydroxychloroquine • Gold • Methotrexate • Ciclosporin • Azathioprine • Sulfasalazine • Cyclophosphamide • Leflunomide • Penicillamine • Biologic therapies, e.g. rituximab, infliximab, etanercept, adalimumab Use only under consultant supervision. d disease progression by modifying the immune response and inflammation. Used individually or in combination, they are now started very early in the disease (i.e. first 3–6 mo)— hence the need for early referral. DMARDs can take several months to show any effect. Before starting check baseline U&E, Cr, eGFR, LFTs, FBC, and urinalysis. Side effects and monitoring—see Table15.11. • Results requiringaction • Platelets 105fL, check B12/folate

Intramuscular gold (Myocrisin®) 50mg monthly

FBC and urinalysis at the time of each injection CXR within 1y of start of treatment

Ask patients to report: Symptoms/signs of infection— especially sore throat, bleeding/bruising, breathlessness/cough, mouth ulcers/metallic taste, or rashes

Penicillamine

FBC, urinalysis 2-weekly for 3mo and 1wk after any i dose Then monthly

Altered taste (can be ignored), rash

FBC and LFT weekly for 6wk, then every 2wk until dose/ monitoring stable for 6wk Then monthly

GI side effects, rash, bone marrow suppression Avoid live vaccines

500–750mg/d maintenance Azathioprine 1.5–2.5mg/kg/d maintenance

• If allopurinol is co-prescribed, d dose to 25% of the original Ciclosporin

FBC and LFT monthly until dose/monitoring stable for 3mo, then every 3mo U&E, Cr/eGFR every 2wk until dose stable for 3mo, then monthly Lipids 6-monthly

Rash, gum soreness, hirsutism, renal failure/i Cr (if i by >30% from baseline, withhold and discuss with rheumatologist), i BP Monitor BP

Hydroxychloroquine 200–400mg/d maintenance

Baseline eye check and annual check of visual symptoms and visual acuity

Rash, GI effects, ocular side effects (rare)

Leflunomide 10–20mg/d maintenance

FBC and LFT monthly for 6mo then, if stable every 2mo

Rash, GI, i BP, i ALT Check weight and BP at each review

1.25mg/kg bd maintenance

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The spondylarthropathies A group of inflammatory rheumatic diseases characterized by predom­ inant involvement of axial and peripheral joints and entheses (areas where tendons, ligaments, or joint capsules attach to bone). Includes: • Ankylosing spondylitis • Psoriatic arthritis • Reactive arthritis and Reiter’s syndrome • Behçet’s disease • Arthritis that accompanies inflammatory bowel disease • Whipple’s disease (b p.407) Sacroiliitis and spondylitis occur with all of them, and they are all associated with the HLA B27 genotype.

Ankylosing spondylitis (AS) Prevalence 1:2,000. 4:5 82½:1. 95% HLA B27+ve—prevalence in a population mirrors the frequency of the HLA B27 genotype. Risk of developing AS if HLA B27+ve81:3. Presentation  Typically presents with morning back pain/stiffness in a young man. Progressive spinal fusion (ankylosis) leads to d spinal movement, spinal kyphosis, sacroiliac (SI) joint fusion, neck hyperextension, and neck rotation. Other features: • Crohn’sorUC • d chest expansion • Heart disease—carditis, aortic • Chestpain regurgitation, conduction defects • Hip and knee arthritis • Osteoporosis • Plantar fasciitis and other • Psoriaformrashes enthesopathies • Iritis Tests • Blood FBC—normochromic or microcytic hypochromic anaemia, i ESR (may be normal), rheumatoid factor is usually–ve • X-ray Initial signs are widening of the SI joints and marginal sclerosis— later, SI joint fusion and a ‘bamboo spine’ (vertebral squaring/fusion) Management  Aims to d inflammation, pain, and stiffness; alleviate systemic symptoms, e.g. fatigue; and slow or stop long-term progression of the disease. Exercise helps back pain. NSAIDs (e.g. naproxen 500mg bd) also help pain. Refer to a rheumatologist early for confirmation of diagnosis, education, disease-modifying drugs (b p.517), and advice on appropriate exercise regimes to maintain mobility.

Psoriatic arthritis Inflammatory arthritis associated with psoriasis

(740% psoriasis patients. 4 = 5). 75% patients have a pre-existing history of psoriasis before the arthropathy; in 15% the rash appears simultaneously with the joint symptoms; in 10% the arthritis precedes the skin changes. Presentation is variable. Patterns include: • Distal arthritis DIP joint swelling of hands/feet, nail dystrophy ± flexion deformity. Sausage-shaped fingers are characteristic of psoriatic arthritis affecting thehand • Rheumatoid-like polyarthropathy Similar to rheumatoid arthritis but less symmetrical and rheumatoid factor is–ve

The spondylarthropathies

• Mutilans Associated with severe psoriasis. Erosions in small bones of hands/feet l progressive deformity • Ankylosing spondylitis/sacroiliitis Usually HLA B27+ve Investigations  WBC—usually i; ESR/CRP—usually i; rheumatoid factor–ve; X-ray appearances can be diagnostic. Management  Education; physiotherapy; NSAIDs. Refer to rheumatology for confirmation of diagnosis, advice on management, and disease-modifying drugs (b p.517). Medication, e.g. methotrexate, may improve both skin and musculoskeletal symptoms.

Reactive arthritis Often asymmetrical aseptic arthritis in ≥1 joint.

Occurs 2–6wk after bacterial infection elsewhere—e.g. gastroenteritis (Salmonella, Campylobacter), GU infection (chlamydia, gonorrhoea). i incidence in HLA B27+ve individuals. Management  NSAIDs, physiotherapy, and steroid joint injections. Recovery usually occurs within months. Aminority develop chronic arthritis requiring disease-modifying drugs. Refer to rheumatology. Reiter’s syndrome  Polyarthropathy, urethritis, iritis, and a psoriaform rash. Affects men with HLA B27 genotype. Commonly follows genito-urinary or bowel infection. Joint and eye changes are often severe. Refer for specialist management. H.C. Reiter (1881–1969)—German public health physician.

Behçet’s disease Multi-organ disease of unknown cause (although

thought to be infective). 4:5 8 2:1. Clinical picture (only some features):arthritis; ocular symptoms and signs—pain, d vision, floaters, iritis; scarring, painful ulceration of mouth and/or scrotum; colitis; meningoencephalitis. Refer to GUM clinic, ophthalmologist or general physician depending on symptom cluster. Treatment is usually with steroids ± azathioprine or ciclosporin. Topical steroids may be useful for ulcers. H. Behçet (1889–1948)—Turkish dermatologist.

Enteropathic spondylarthropathy Oligoarticular or polyarticular arthritis linked to inflammatory bowel disease. Presentation is variable and includes:sacroiliitis, plantar fasciitis, inflammatory spinal pains, and other enthesitides (insertional ligament/tendon inflammation). Arthritis may evolve and relapse/remit independently of bowel disease. Management  NSAIDs may help joint pain but aggravate bowel disease. Refer to rheumatology for confirmation of diagnosis, advice on management, and disease-modifyingdrugs.

Information and support for patients National Ankylosing Spondylitis Society (NASS) F 020 8948 9117 Mwww.nass.co.uk Psorasis and Psoriatic Arthritis Alliance (PAPAA) F 01923 672837 Mwww.papaa.org Arthritis Research UK F 0300 790 0400 M www.arthritisresearchuk.org

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Crystal-induced arthritis Hyperuricaemia  Increased serum uric acid. Causes: • Drugs Cytotoxics; thiazides; ethambutol • i cell turnover Lymphoma; leukaemia; psoriasis; haemolysis; muscle necrosis • d excretion Primary gout; chronic renal failure; lead nephropathy; hyperparathyroidism In addition  Associated with i BP and hyperlipidaemia. Urate may also be i in disorders of purine synthesis, e.g. Lesch–Nyhan syndrome.

Acute gout  Intermittent attacks of acute joint pain due to deposition of uric acid crystals. Prevalence: 3–8/1,000. i with age; 4:5 85:1. Predisposing factors:

• FH • Obesity • Excess alcohol intake • High-purinediet

• Diuretics • Acute infection • Ketosis • Surgery • Plaque psoriasis

• Polycythaemia • Leukaemia • Cytotoxic treatment • Renal failure

Presentation  Painful swollen joint (big toe, feet, and ankles most commonly); red skin which may peel ± fever. Can be polyarticular— especially in elderly 5. May mimic septic arthritis. Investigation • Blood i WCC; i ESR; i blood urate (but may be normal) • Microscopy of synovial fluid Not usually required—reveals sodium monourate crystals on polarized light microscopy • X-rays Not usually required—show soft tissue swelling only, unless severe disease when an erosive pattern isseen Management  Resolves in 30mm/h) or i CRP) 0 Diagnosis can be made without i inflammatory markers if classical clinical picture and rapid response to steroid treatment Diagnosis of GCA  A person may be regarded as having GCA if ≥3 of the following criteria aremetG: • Age≥50y • New headache—unilateral throbbing headache, facial pain, scalp tenderness, e.g. on brushing hair and/or jaw claudication (i likelihood of visual symptoms) • Temporal artery abnormality (tenderness, thickening, d pulsation) • i ESR >50mm/h (oriCRP) • Abnormal temporal artery biopsy compatible withGCA 0 Visual symptoms (amaurosis fugax, diplopia, or sudden loss of vision) are early complications of GCA and may be the presenting feature.

Differential diagnosis PMR  • Inflammatory arthritis, e.g.RA • Connective tissue disease/ vasculitis, e.g.SLE • OA • Septic arthritis • Shoulder disease • Neoplasia, e.g. myeloma • Occult sepsis, e.g. endocarditis • Inflammatory myopathy • Fibromyalgia • Endocrinopathy/metabolic bone disease

GCA  • Herpeszoster • Migraine • Intracranial pathology • Other causes of acute vision loss, e.g. amaurosisfugax • Cluster headache • Cervical spondylosis or other cervical spine disease • Sinus/ear disease • TMJpain • Connective tissue disease/ vasculitis

Polymyalgia and giant cell arteritis

Further investigation  Intended to exclude other diagnoses:

• Full blood count—normocytic anaemia may be seen in PMR/GCA • TFTs • Urea and electrolytes/eGFR • Creatinekinase • Liver functiontests • Dipstick urinalysis • Bone profile • Protein electrophoresis (also consider urinary Bence Jones protein) • Rheumatoid factor (consider ANA and anti-CCP antibodiestoo) • Consider CXR and/or hip/pelvis/shoulder/cervical spineX-ray Further management of PMR If typical symptoms/signs management in primary care is appropriate. • Start prednisolone 15mg od—there should be a rapid response (≥70% d of symptoms in 2y

Further managementofGCA • Corticosteroids prevent vascular complications, particularly blindness, and rapidly relieve symptoms (70% improvement in 5 Presents with a purpuric rash over buttocks and extensor surfaces. Platelet count is normal Often follows a respiratory infection Other features: urticaria, nephritis, joint pains, abdominal pain (may mimic acute abdomen)

Refer to paediatrics for confirmation of diagnosis Most recover fully without treatment over a few months

Uncommon in the UK. 4:58 4:1 Peak incidence in middle age Multisystem necrotizing vasculitis l aneurysms of medium-sized arteries Presents with: tender subcutaneous nodules along the line of arteries, coronary arteritis, i BP, mononeuritis multiplex, renal failure, and gastrointestinal symptoms Sometimes associated with hepatitis B

Refer to rheumatology for angiography to confirm diagnosis and for advice on management Treatment is with control of i BP, high-dose steroids, and cyclophosphamide

Henoch– Schönlein purpura (HSP)

Polyarteritis nodosa (PAN)

(Continued)

Vasculitis

Table15.13  (Cont.) Condition Churg–Strauss syndrome

Features Associated with asthma Affects coronary, pulmonary, cerebral, and splanchnic circulations Skin manifestations and mononeuritis can also occur Diagnosis is based on clinical features and biopsy

Management Refer for specialist treatment with high-dose prednisolone ± cyclophosphamide Avoid leukotriene receptor agonist drugs for control of asthma as may worsen symptoms

Wegener’s granulomatosis

Granulomatous vasculitis Any organ may be involved and symptoms/signs relate to those affected, e.g. mouth ulcers; nasal ulceration with epistaxis/rhinitis; otitis media; cranial nerve lesions; lung symptoms and shadows on CXR; i BP; eye signs (50%) Often long prodrome of ‘limited Wegener’s granulomatosis’— nasal stuffiness, headaches, hearing difficulties, and nose bleeds

Refer to rheumatology/ general medicine for investigation ANCA helps diagnostically and in disease monitoring Treatment is with high-dose steroids, methotrexate, mofetil, and cyclophosphamide

Predominantly affects children 15mm diameter (usually single and painful) i suspicion if poor response to anti-pyretics

Early treatment (6–12wk and symptoms/ signs of depression, consider a trial of antidepressants, e.g. sertraline 50mgod. Chronic fatigue syndrome (CFS, ME)  A debilitating and distressing condition. Prevalence: 0.2–2.6%; 5:4 83:2. Cause is unknown though viral infections (810% after EBV), immunization, chemical toxins (e.g. organophosphates, chemotherapy drugs) have all been implicated. 0 Fatigue must have been present for ≥4mo for adults and ≥3mo for children and young people for a diagnosis of CFS to bemade.

Tiredness and chronic fatigue syndrome

Clinical features Unexplained persistent and/or recurrent fatigue of new/ definite onset, not explained by other conditions and resulting in d activity (often starting 1–2d after mental/physical exertion and lasting >24h) and ≥1of: • Physical/mental exertion makes • General malaise symptomsworse • Dizziness/nausea • Headaches of new type, pattern, • Palpitations without cardiac or severity dysfunction • Multi-site muscle/joint pain • Cognitive dysfunction, e.g. without inflammation impaired concentration/memory • Sorethroat • Tender cervical/axillary LNs • Difficulty with sleeping without enlargement Additional symptoms Must not have pre-dated fatigue. Symptoms may fluctuate or change in nature over time. Include: • Fibromyalgia • Sensitivity to light/ • Postural dizziness • Feelings of dyspnoea sound • Vertigo • Moodswings • Altered temperature • Palpitations • Panic attacks • IBS sensation • Depression • Food intolerance • Paraesthesiae 0 Infection/immunization, drugs, caffeine, alcohol, and stress cause setbacks. • Red flag symptoms that suggest another diagnosis • Signs/symptoms of • Significant weightd cardiorespiratory disease • Localizing/focal • Sleepapnoea neurologicalsigns • Signs/symptoms of inflammatory • Clinically significant lymphadenopathy arthritis or connective tissue disease Children presenting with sustained fatigue of any duration with no obvious cause should always be referred for paediatric review. Reconsider diagnosis if none of the following are present Cognitive difficulties; chronic pain; post-exertional fatigue/malaise; sleep disturbance. Management  Provide support and reassurance—explanation, information ± self-help groups. Avoid exacerbating factors, e.g. caffeine, alcohol. Advise graded exercise and regular, limited rest periods (e.g. 30min 4–5x/d). Treat symptoms, e.g. amitriptyline 10–50mg nocte to help sleep ± relieve headache/neuropathic pain; SSRI for depression. Refer adults if severe symptoms or symptoms persist >6mo. Specialist treatments include CBT and rehabilitation programmes. Prognosis  Variable. 55% of adults have symptoms >6mo. Risk i x3 if history of anxiety/depression. Prognosis in children is better.

Further information NICE Diagnosis and management of CFS/ME in adults and children (2007) M www.nice.org.uk

Information and support for patients ME Association F 0844 576 5326 M www.meassociation.org.uk Action for ME F 0845 123 2314 M www.actionforme.org.uk

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Miscellaneous conditions Neuropathic arthritis Charcot’s disease is a rapidly progressive

degeneration in a joint which lacks position sense and protective pain sensation. Upper limb disease is usually associated with syringomyelia. Lower limb disease is usually associated with diabetic neuropathy or cauda equina lesions. The joint may be very deformed but is usually painless. Treat the underlying condition (e.g. DM). The joint cannot recover, but refer to orthopaedics for advice on stabilization.

Fibromyalgia  Painful, non-articular condition of unknown cause, predominantly involving muscles. Fibromyalgia is common and often results in significant disability. Peak age 40–50y—90% female. Diagnostic criteria • History of widespread pain (defined as pain on both left and right sides, above and below the waist, together with axial skeletal pain, e.g. neck or back pain), in combinationwith: • Pain in ≥11 out of 18 tender sites (see Figure 15.5) on digital palpation Other clinical features • Clinical findings are unremarkable • Pain is worsened by stress, cold, and activity and associated with generalized morning stiffness; analgesics, NSAIDs, and local physical treatments are ineffective and may worsen symptoms • Sleep patterns are poor—patients tend to wake exhausted and complain of poor concentration; anxiety and depression scores arehigh • Associated symptoms include unexplained headache, paraesthesiae of hands/feet, urinary frequency, and abdominal symptoms Investigation  Exclude other causes of pain and fatigue (e.g. hypothyroidism, SLE, Sjögren’s, psoriatic arthritis, inflammatory myopathy, hyperparathyroidism, osteomalacia)—check FBC, ESR, TFTs, U&E, eGFR, Ca2+, CK, PO43–, ANA, RhF, and immunoglobulins. Management  Multidisciplinary approach is helpful. • Be supportive—reassurance that there is no serious pathology, explanation and information arevital • Low-dose amitriptyline 25–75mg nocte may help with sleep/pain. SSRI, e.g. sertraline 25–50mg od, may help anxiety, depression, and sleep— stop if no improvement after a month’strial • Graded exercise regimes improve pain, lethargy, mood, and malaise • Counselling/learning coping strategies/CBT can be beneficial • X Some patients benefit from injection of hyperalgesic trigger points with steroid or acupuncture to trigger points

Hypermobility Children/young adults with lax joints; R), sometimes termed slipped rib syndrome. Management  Explanation and reassurance that nothing serious is happening; simple OTC analgesia, e.g. ibuprofen 400mg tds. If pain persists, local steroid or marcaine injections can be helpful. If not settling consider referral to rheumatology. A.Tietze (1864–1927)—German surgeon.

Further information European League Against Rheumatism (EULAR) Evidence-based recommendations for the management of fibromyalgia syndrome(2007).

Patient information and support Arthritis Research UK F 0300 790 0400 M www.arthritisresearchuk.org Fibromyalgia Association UK F 0844 887 2444 M www.fibromyalgia-associationuk.org Hypermobility Syndrome Association (HMSA) M www.hypermobility.org

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Neurology Reflexes and muscle power  534 Cranial nerve lesions  536 Neuropathy  538 Polyneuropathy  542 Walking problems  544 Other movement problems  546 Speech problems  548 Fits, faints, and funny turns  550 Assessment of headache  552 Migraine  554 Other headaches and facial pain  556 Raised intracranial pressure  558 Intracranial bleeds  560 Acute stroke  562 Management after stroke  564 Parkinsonism and Parkinson’s disease  566 Multiple sclerosis  568 Motor neurone disease and CJD  570 Spinal cord conditions  572 Epilepsy  574 Management of epilepsy  576 Muscle disorders  578 Other neurological syndromes  580 Neurological rehabilitation problems  582 Neurological assessment scales  584

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Reflexes and muscle power Automatic responses. The reflex arc goes from the stimulus via a sensory nerve to the spinal cord and then back along a motor nerve to cause muscle contraction, without brain involvement.

Key reflexes  See Table16.2. Record whether absent, present with reinforcement, normal, or brisk 9 clonus.

Absent or dreflex  Implies a breach in the reflex arcat: • Sensory nerve or root, e.g. neuropathy, spondylosis • Anterior horn cell, e.g. MND,polio • Motor nerve or root, e.g. neuropathy, spondylosis • Nerve endings, e.g myasthenia gravis,or • Muscle, e.g. myopathy

ireflex  Implies lack of higher control—an upper motor neurone lesion, e.g. post-stroke. Clonus  Rhythmic involuntary muscle contraction due to abrupt tendon stretching, e.g. by dorsiflexing the ankle—associated with an UMN lesion. Reinforcement  Method of accentuating reflexes. Use if a reflex seems absent. Ask patients to clench their teeth (to reinforce upper limb reflexes) or clench their hands and pull in opposite directions (to accentuate lower limb reflexes). This effect only lasts ~1s, so ask patients to perform the manoeuvre simultaneously with the tap from the tendon hammer. Testing for muscle power  See Table 16.1 Table16.1  Quick screening test for musclepower Joint

Movement

Nerve roots Joint

Shoulder

Abduction

C5,6

Adduction

C6–8

Elbow

Flexion

C5,6

Extension

C7,8

Flexion

C7,8

Extension

C6,7

Wrist Fingers

Flexion

C8

Extension

C7

Abduction

T1

Movement

Nerve roots

Hip

Flexion

L1–3

Extension

L4,5& S1

Knee

Flexion

L5& S1

Extension

L3,4

Ankle Toes

Dorsiflexion

L4,5

Plantarflexion

S1,2

Extensors

L5,S1

Flexors

S2

0 Test proximal muscle power by asking the patient to sit from lying, pull you towards him/herself, or rise from squatting.

Reflexes and muscle power

Table16.2  Key reflexes and nerve roots involved Reflex

Test

Expected result

Nerve roots

Jaw

Ask the patient to let his mouth open slightly. Place a finger on the chin and tap the finger with a tendon hammer

Contraction of masseters and closure of mouth

Vth cranial nerve

Gag

Contraction of the Touch the back of the patient’s soft palate pharynx on each side with a spatula, If absent, ask the patient whether he can feel the spatula— if he can, then Xth nerve palsy

Biceps

Tap a finger placed on the biceps tendon by letting the tendon hammer fall on it

Contraction of the biceps + elbow flexion

C5, C6

Supinator

Tap the lower end of the radius just above the wrist with the tendon hammer

Contraction of brachioradialis + elbow flexion

C5, C6

Triceps

Support elbow in flexion with one hand. Tap the triceps tendon with a tendon hammer held in the other hand

Contraction of triceps + elbow extension

C7, C8

Knee

Contraction of Support the knees so that relaxed and slightly bent. Let the quadriceps + extension of knee tendon hammer fall onto the infrapatellar tendon

Ankle

S1 Externally rotate the thigh and flex Contraction of the knee. Let the tendon hammer gastrocnemius + plantar flexion of the ankle fall onto the Achilles tendon

Abdominal

Lightly stroke the abdominal wall diagonally towards the umbilicus in each of the four abdominal quadrants

Abdominal wall contractions. When absent can be normal or indicate UMN or LMN lesion

Cremaster

4 patients only. Pre-warn the patient. Stroke the superior and medial aspect of the thigh in a downwards direction

L1 Contraction of cremasteric muscle l raising of scrotum and testis on the side stroked. Absent in UMN and LMN lesions

Anal

Scratch the perianal skin

Reflex contraction of S4, S5 the external sphincter. Absent in UMN and LMN lesions

Plantar

Pre-warn the patient. Run a blunt object up the lateral side of the sole of the foot, curving medially before the MTP joints

Flexion of big toe (if >1y old). Extension implies UMN lesion

IXth/Xth cranial nerve

L3, L4

T7–T12

S1

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Cranial nerve lesions Table 16.3 summarizes cranial nerve lesions and their causes. Figure 16.1 shows the cutaneous innervation of the head and neck. Cranial nerves may be affected at any point from the nerve nucleus within the brainstem to the point of innervation. Think systematically about the level of the lesion. Potentialsites: • Muscle • Neuromuscular junction • Along the course of the nerve outside the brainstem • Within the brainstem 0 Any cranial nerve may be affected by DM, MS, tumours, sarcoid, vasculitis or syphilis and >1 nerve may be affected by a lesion. Refer according to cause to ENT, ophthalmology, or neurology. Table16.3  Cranial nerve lesions and theircauses Nerve

Clinical test

Causes

I Olfactory Smell—test each nostril for the ability to differentiate different smells

Trauma, frontal lobe tumour, meningitis

II Optic

Acuity—Snellen chart Visual fields—compare with your own visual fields by standing directly in front of the patient with your head at the same level as theirs Pupils—size, shape, reaction to light, and accommodation Ophthalmoscopy—darken room, dilate pupil with 1 drop tropicamide 0.5% if needed, view optic disc (? pale, swollen), follow each vessel outwards to view each quadrant, track outwards to check lens and cornea

Monocular blindness—lesion in one eye or optic nerve (e.g. MS,giant cell arteritis) Bitemporal hemianopia— optic chiasm compression, e.g. pituitary adenoma, craniopharyngioma, internal carotid artery aneurysm hom*onymous hemianopia— affects half the visual field on the side opposite the lesion. Caused by lesion beyond the optic chiasm, e.g. stroke, abscess, tumour

III

Ptosis, large pupil, eye looks down- and outwards 0 Diplopia from a 3rd nerve lesion may cause nystagmus

DM, giant cell arteritis, syphilis, posterior communicating artery aneurysm, idiopathic If pupil normal size, results fom DM or other vascular cause

IV

Diplopia on looking down and in; may compensate by tilting head

Rare in isolation. May occur as a result of trauma to the orbit

V Trigeminal

Motor—open mouth. Jaw deviates to the side of the lesion Sensory—corneal reflex lost first. Check all three divisions

Motor—bulbar palsy (b p.549), acoustic neuroma Sensory—trigeminal neuralgia (b p.549); herpes zoster; nasopharyngeal carcinoma

VI

Horizontal diplopia on looking outwards

MS, pontine CVA, i ICP (continued)

Cranial nerve lesions Table16.3  (Cont.) Nerve VII Facial

Clinical test Causes facial weakness and droop Ask to raise eyebrows, show teeth, puff out cheeks. •  LMN lesion: all one side of face affected •  UMN lesion: lower two-thirds of the face affected only

Causes LMN—Bell’s palsy, polio, otitismedia, skull fracture, cerebello pontine angle tumour, parotid tumour, herpes zoster (Ramsay Hunt syndrome b p.538) UMN—stroke, tumour

VIII Vestibuloauditory

Auditory—ask to repeat a number whispered in 1 ear whilst you block the other Vestibular—ask about balance, check for nystagmus (bp.950)—ask patient to fix on finger ¾ m away—check gaze upwards, downwards, lateral (both directions), keeping finger 1 peripheral nerve is involved, the term mononeuritis multiplex is used. Causes: DM, sarcoid, cancer, PAN, amyloid, leprosy.

Common mononeuropathies  See Table16.4. Bell’s palsy  Facial palsy without other signs. Unknown cause—possibly

viral. Peak age: 10–40y. 4 = 5. Lifetime incidence: 71:65. Affects left and right side of the face equally often. Usually sudden onset—may be preceded by pain around the ear. Other possible symptoms: facial numbness; dnoise tolerance; disturbed taste on the anterior part of the tongue.

Management  770% recover completely; 13% have insignificant sequelae; the remainder have permanent deficit. 85% improve in 4. Known precipitants: fright (e.g. during venesection) or emotion. Exclude other reasons for loss of consciousness. No treatment needed—reassure.

Dizziness and giddiness  Distinguish between true vertigo (the illusion of rotatory movement—the room spinning) and a feeling of unsteadiness or light-headedness: • Vertigo bp.950 • Imbalance Implies difficulty in walking straight, e.g. from disease of peripheral nerves, posterior columns, or cerebellum • Faintness The feeling of being about to pass out. Associated with some seizure disorders and a variety of non-neurological conditions (e.g. postural hypotension, vasovagal fainting; hyperventilation; hypoglycaemia; arrhythmias; cough syncope). Sometimes >1 element coexists Hyperventilation and panic attacks  b p.1120 0 Usually history is diagnostic but occasionally, seizures of temporal lobe origin may have similar symptomatology. Hypoglycaemia  Affects patients with DM—particularly those tak-

ing insulin or oral hypoglycaemic agents. Produces autonomic changes, e.g. pallor, sweating and tachycardia, and behavioural changes (confusion, altered personality). If action is not taken to i blood sugar, coma ± fitting ensues—b p.1100.

Abnormal perceptions  (e.g hallucinations—b p.988).

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Assessment of headache Common presenting complaint. The skill lies in deciding which headaches are benign, needing no intervention, and which require action.

History • Is there >1 type of headache? Take a separate history foreach. • Time When did the headaches start? New or recently changed headache calls for especially careful assessment. How often do they happen? Do they have any pattern? (e.g. constant, episodic, daily) How long do they last? Why is the patient coming to the doctor now? Aheadache diary over >8wk may help if long-standing headaches • Character Nature/quality, site, and spread of the pain. Associated symptoms, e.g. nausea/vomiting, visual disturbance, photophobia, neurological symptoms • Cause Predisposing and/or trigger factors; aggravating and/or relieving factors; relationship to menstrual cycle; family history • Response Details of medication used (type, dose, frequency, timing). What does the patient do, e.g. can the patient continuework? • Health between attacks Do headaches go completely or is the patient unwell between attacks? Other past/current medical problems • Anxieties and concerns Of the patient/family

Examination  In acute, severe headache, examine for fever and purpuric

skin rash. In all cases check BP, brief neurological examination including fundi, visual acuity, and gait, palpation of the temporal region/sinuses for tenderness, and examination of the neck. In young children, measure head circumference and plot on a centilechart.

• Redflags • Fever and worsening headache ±purpuric rash/meningism • Thunderclap headache (reaching peak intensity in1y

b p.554 b p.556

Exertional or coital headache

Suggested by history of association

NSAID or propranolol before attacks

Trigeminal neuralgia

Intense stabbing pain lasting seconds in trigeminal nerve distribution

b p.557

Glaucoma

Red eye, haloes, d visual acuity, pupil abnormality

b p.976

Subacute Giant cell headache arteritis

>50y, scalp tenderness, i ESR, rarely d visual acuity

b p.524

Tension type Chronic headache headache

Band around the head, stress, low mood

b p.556

Cervicogenic headache

Unilateral or bilateral; band from neck to forehead; scalp tenderness

b p.474

Medication overuse

Rebound headache on stopping analgesics

b p.557

i intracranial pressure

Worse on waking/ sneezing, d pulse, i BP, neurological signs

b p.558

Paget’s disease

>40y, bowed tibia, i alk phos

b p.504

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Migraine Migraine affects 15% of the UK population. 4:5 81:3. One in three experiences significant disability. Caused by disturbance of cerebral blood flow under the influence of5HT.

Aura  Occurs with or without headache. Symptoms arise over ≥5min and last 5–60min before resolving completely. Diagnoseif: • Visual symptoms, e.g. flickering lights, spots, lines; partial loss ofvision • Sensory symptoms, e.g. numbness; paraesthesiaand/or • Speech disturbance Atypicalaura Consider referral for further investigation if:motor weakness; double vision; visual symptoms affecting only one eye; poor balance or dlevel of consciousness. Migraine headache  Moderate to severe unilateral or bilateral throbbing/pulsating headache that lasts 4–72h (1–72h in children) and prevents usual activities. May occur with or without aura and be associated with nausea/vomiting ± i sensitivity to light/noise. • Episodic Occurs on 3mo History, examination, and differential diagnosis  bp.552

Management of an acute attackN  Combination therapy with: • Triptan (e.g. sumatriptan 50–100mg po)—choice depends on cost. Not effective if taken before the headache develops. Stops 70–85% attacks. Start with lowest dose and i as needed. If consistently ineffective try an alternative triptan. Consider nasal triptan as first-line if aged12–17y • NSAID (e.g. naproxen 500mg bd) or paracetamol (1g qds) ±antiemetic (e.g. prochlorperazine 5mg, metoclopramide 10mg, or domperidone 10–20mg)—even if no nausea/vomiting If oral preparations are ineffective/not tolerated, offer metoclopramide 10mg pr or buccal prochlorperazine 3–6mg and consider adding a non-oral NSAID (e.g. diclofenac 100mg pr) or triptan (e.g. sumatriptan 20mg nasal spray or 6mg sc). • Do not offer ergots or opioids for the acute treatment of migraine. Treatment of recurrence within the sameattack Repeat symptomatic treatments within their dose limitations—pre-emptively if recurrence is usual/expected. If using triptans, a second dose may be effective, but repeated dosing can cause rebound headache. Naratriptan and eletriptan are associated with relatively low recurrencerates. Management of chronic migraineG  Aims to control symptoms and minimize impact on the patient’s life. Cure is not a realisticaim. Trigger factors  Half have a trigger for their migraine. Consider: • Psychological factors Stress/relief of stress; anxiety/depression; extreme emotions, e.g. anger orgrief • Environmental factors Loud noise, bright/flickering lights, strong perfume, stuffy atmosphere, VDUs, strong winds, extreme heat/cold

Migraine

• Food factors Lack of food/infrequent meals; foods containing monosodium glutamate, caffeine, and tyramine; specific foods, e.g. chocolate, citrus fruits, cheese; alcohol, especially redwine. • Sleep Overtiredness (physical/mental); changes in sleep patterns (e.g.late nights, weekend lie-in, shift work, holidays); long-distance travel • Health factors Hormonal changes (e.g. monthly periods, CHC, HRT, the menopause); i BP; toothache or pain in the eyes, sinuses, or neck; unaccustomed physical activity Assessing severity  Assessment scales, e.g Migraine Disability Assessment Score (MIDAS—see Box 16.1 b p.585), can be useful in assessing impact of symptoms on daily life and monitoring response to treatment. General measures  Reassure. Instruct about management of acute attacks. Adiary can be used to identify trigger factors, assess headache frequency, severity, medication usage/overusage, and response to treatment. Avoid trigger factors where possible. Give advice on relaxation techniques and stress management. Do not offer CHC to women with migraine, especially if aura (b p.753). ProphylaxisN  Consider if ≥4 attacks/mo or severe attacks. d attacks by 750%. Try a drug for 2mo before deeming it ineffective. If effective, continue for 6mo then review to consider d dose slowly before stopping. • 1st-line Propranolol S/R 80–160mg od/bd or topiramate 25–50mg od/ bd—start at low dose and i dose every 2–4wk; 0 Topiramate is teratogenic and may interact with hormonal contraception • 2nd-line Gabapentin (up to 1200mg/d in divided doses) or acupuncture (up to 10 sessions over 5–8wk) • 3rd-line Botulinum type Atoxin may be helpful for patients who have chronic migraine, do not have medication-overuse headache and have not responded to ≥3 different prophylactic medicationsN Alternative therapies  Riboflavin 400mg od may d frequency/intensity of headachesN; feverfew 200mg daily mayd symptoms after 6wkuseC.

Menstrual migraineN  Suspect if migraine occurs from 2d before to 3d after start of period on at least 2 out of 3 consecutive months (use headache diary). If predictable menstrual-related migraine that does not respond to standard acute treatment, consider frovatriptan (2.5mg bd) or zolmitriptan (2.5 mg bd/tds) on the days that migraine is expected. 0 >1 type of headache may coexist—50% migraine sufferers develop tension type headache resulting in background pain between attacks. Consider each separately.

Further information

NICE F www.nice.org.uk • Headaches in young people and adults(2012) • Migraine (chronic)—botulinum toxin type A(2012)

Patient information and support

Migraine Action Association F0116 275 8317 M www.migraine.org.uk Migraine Trust F 020 7631 6970 M www.migrainetrust.org

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Other headaches and facialpain Assessment and differential diagnosis of headache  bp.552 Migraine  bp.554 Chronic daily headache  Prevalence 4%. Defined as any headache

that occurs >15d/mo. Common causes: tension-type headache, cervicogenic headache (b p.474), medication-overuse headache, migraine, errors of refraction (usually headache is mild, frontal, in the eyes themselves, and absent on waking). Treat the cause (may be>1).

Tension type headacheN  Associated with stress and anxiety and/or

functional or structural abnormalities of the head or neck. Prevalence82%. 5:4 82:1. Symptoms begin aged 1 TIA in 3x normal)—1point •  Abnormal renal function (dialysis, Cr >200micromol)—1point •  Stroke history—1point •  Prior major bleed or predisposition to bleeding—1point •  Labile INR (10y; fit-free2y—discuss the possibility of withdrawing medication, if appropriate. For women of reproductive age give contraception (b p.756) and pre-conception advice (b p.829). Re-refer  For review by a neurologist or epilepsy nurse specialist if:

• Control is poor or drugs are causing unacceptable side effects • Seizures have continued despite medication for >2y or on twodrugs • Pointers to a previously unsuspected cause for the fitsappear • Concurrent illness (physical or psychiatric) complicates management • For pre-conceptual advice or to discuss withdrawal of medication

Table16.13  Commonly used drugs in epilepsy. Stress the importance of concordance. Start at a low dose, and i dose until fits are controlled or side effects occur. Use monotherapy wherever possible—two drugs i toxicity and side effects. Polytherapy offers no advantage over monotherapy for 90% patients. Prescribe by brand name—generic prescribing may lead to changing of brand. Changing brand carries 10% risk of worsening of seizure control. Type of epilepsy

Absence Myoclonic Tonic clonic Partial ± 2o generalized

Ethosuximide

Sodium valproate

  1 

 

300mg bd 200mg/d at 3-day intervals

100–200mg od or bd 100mg/d at weekly intervals

500mg od 250mg/d at weekly intervals

Usual daily dose Common/important side effects

1–1.5g od Blood dyscrasias3, sedation, nausea, vomiting, dizziness, ataxia

      4   5   1 2 3

Lamotrigine

Clonazepam

 

 

25mg od for 2wk2 From starting dose to 50mg od for 2wk, then i by 50mg/d at weekly intervals 500mg–1g bd 200–1200mg 100–200mg Pancreatitis, liver Blood dyscrasias3, Blood dyscrasias3, rash, toxicity4, blood rash, liver toxicity4, fever, influenza-like dyscrasias3, sedation, nausea, sedation, diplopia, symptoms, drowsiness, tremor, weight i, hair dizziness,fluid retention, or worsening of seizure thinning, ankle swelling d Na+5 control

Drug of choice for primary syndromes of generalized epilepsy. Starting dose is different if used in association with other epileptics—seeBNF. Check FBC if bruising, mouth ulcers, or symptoms of infection (sore throat, fevers). Warn about symptoms of liver disease. Check LFTs soon after starting and at review. Monitor U&E at regular review.

1mg nocte for 4d i according to response over 2–4wk

4–8mg nocte Drowsiness/fatigue, amnesia/confusion/ restlessness, muscle hypotonia, co-ordination problems, dependence, and withdrawal

Management of epilepsy

Adult starting dose Incremental dose

Carbamazepine

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Muscle disorders Symptoms  Muscle weakness, fatigability. Pain at rest suggests inflammation—pain on exercise, ischaemia, or metabolic myopathy.

Signs  Look for associated systemic disease.

• Myotonia—delayed muscular relaxation after contraction, e.g. difficulty letting go after gripping something • Local muscular tenderness or firm muscles—may be due to infiltration of muscle with connective tissueorfat • Fasciculation—spontaneous, irregular, and brief contractions of part of a muscle; suggests LMN disease, e.g.MND • Lumps—tumours are rare; lumps may be due to tendon rupture, haematoma, or herniation of muscle through fascia Muscular dystrophies  Group of genetic disorders characterized by progressive degeneration and weakness of some muscle groups. Myotonic dystrophy (dystrophia myotonia)  Autosomal dominant inheritance—abnormal DMPK gene on chromosome 19. Presents at any age. Symptoms vary from mild to severe and may include: • Muscle symptoms—weakness and myotonia, particularly involves face, eyelids, jaw, neck, forearms/hands, lower legs/feet. Can affect speech and result in a lack of facial expression • Respiratory symptoms—weakness of respiratory muscles l poor night-time sleep, daytime sleepiness, headaches, and difficulty waking; aspiration l recurrent chest infections • Eye symptoms—cataract (may be the only problem) andptosis • Reproductive problems—infertility as a result of atrophy of the testes and problems in labour due to uterine muscle weakness • Learning difficulty and behavioural problems • Digestive symptoms—swallowing difficulty, abdominal pain, constipation/diarrhoea, gallstones • Cardiac arrhythmias—annual ECG is advisable • Endocrine abnormalities, e.g.DM • Anaesthetic problems—pre-warn anaesthetist/surgeon prior to surgery Prognosis is variable depending on severity of symptoms. Refer to confirm diagnosis and for advice on management/genetic counselling. duch*enne’s muscular dystrophy  Sex-linked recessive inheritance means almost always confined to boys. 30% of cases are due to spontaneous mutation. Investigation shows markedly i CK (>40x normal). Presents typically at 74y with progressively clumsy walking. Few survive to >20y old. Refer for confirmation of diagnosis and ongoing specialist support. Genetic counselling is important. G.B.A. duch*enne (1807–75)—French neurologist. Patient information and support Myotonic Dystrophy Support Group F 0115 987 0080 M www.myotonicdystrophysupportgroup.org Muscular Dystrophy Campaign F 0800 652 6352 M www.muscular-dystrophy.org

Muscle disorders

Toxic myopathies  Certain drugs can cause myopathy including:

• Alcohol • Ciclosporin • Steroids • Labetalol • Cocaine • Chloroquine • Cholesterol-lowering • Heroin • Zidovudine drugs (including statins) • PCP • Vincristine Management  Stop the implicated drug immediately. If symptoms do not resolve, refer for confirmation of diagnosis and management advice. Acquired myopathy of lateonset  Often a manifestation of systemic disease, e.g. thyroid disease (especially hyperthyroidism), carcinoma, Cushing’s disease. Investigate to find the cause. Treat the cause if found else refer for further investigation. Polymyositis  Insidious, symmetrical, proximal muscle weakness due to muscle inflammation. Dysphagia, dysphonia, and/or respiratory muscle weakness may follow. 25% have a purple rash on cheeks, eyelids, and other sun-exposed areas (dermatomyositis) ± nail fold erythema. CK levels are i. Associated with malignancy in 10% of patients >40y.Refer.

PoliomyelitisND  Acutepolio  Spread: droplet or faecal-oral. Incubation: 7d. Presents with 2d flu-like prodrome then fever, tachycardia, headache, vomiting, stiff neck, and unilateral tremor (‘pre-paralytic stage’). 65% who experience the pre-paralytic stage go on to develop paralysis (myalgia, LMN signs ± respiratory failure). Management: supportive—admit to hospital. 10y and adults Three doses of 3-part vaccine (Td/IPV), each 1mo apart. Give booster doses after 3y and10y • Booster doses for travel Not required unless at special risk, e.g. travelling to endemic/epidemic area or healthcare workers. Boosters of Td/IPV are then given every10y Late effects ofpolio 20–30y after initial infection some patients develop new symptoms often triggered by a period of immobilization: • i muscle weakness and fatigue • Pain in muscles andjoints • Respiratory difficulties (particularly in those who spent some time in an iron lung ventilator)—may present with symptoms relating tosleep Once other causes are excluded, treatment is supportive. Motor neurone disease  bp.570 Myasthenia gravis/Lambert–Eaton syndrome  bp.548

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Other neurological syndromes von Recklinghausen’s disease (type 1 neurofibromatosis; NF1) 

Autosomal dominant trait. Criteria for diagnosis:≥2of: • ≥6 café-au-lait patches (flat, coffee-coloured patches of skin seen in first year of life, increasing in number and size with age) >5mm (pre-pubertal) or >15mm (post-pubertal) • ≥2 neurofibromas: • Dermal neurofibromas—small violaceous skin nodules which appear after puberty • Nodular neurofibromas—subcutaneous, firm nodules arising from nerve trunks (may cause paraesthesiae if compressed) or a plexiform neurofibroma which appears as a large subcutaneous swelling • Freckling in axilla, groin, neck base, and submammary area (women). Present by age10y • ≥2 Lisch nodules—nodules of the iris only visible with a slitlamp • Distinctive bony abnormality specific to NF1, e.g. sphenoid dysplasia • First-degree relative withNF1 Management  Ongoing specialist management is essential. Complications  Affect 1 in 3 patients: • Pseudoarthrosis • Mild learning disability • i BP (6%)—due to renal artery • Short stature stenosis or phaeochromocytoma • Macrocephaly • Malignancy (5%)—optic glioma • Nerve root compression or sarcomatous change of • GI bleeding or obstruction neurofibroma • Cystic bonelesion • Epilepsy (slighti) • Scoliosis F.D.von Recklinghausen (1833–1910)—German pathologist.

Type 2 neurofibromatosis (NF2) Much rarer than type 1. Auto­

somal dominant inheritance.

Diagnosis  Oneof: • Bilateral vestibular schwannoma (acoustic neuroma—sensorineural hearing loss, vertigo ± tinnitus) • First-degree relative with NF2 and either a unilateral vestibular schwannoma or ≥1 neurofibroma, meningioma, glioma, schwannoma, or juvenile cataract Management  Screen at-risk patients with annual hearing tests. Once diagnosis is made, specialist neurosurgical management is needed. Complications  Schwannomas of other cranial nerves, dorsal nerve roots, or peripheral nerves; meningioma (45%); other gliomas (less common).

Ekbom’s syndrome (restless legs syndrome)  The patient (who

is usually in bed) is seized by an irresistible desire to move his/her legs in a repetitive way accompanied by an unpleasant sensation deep in the legs. Sleep disturbance is common, as is +ve FH. Cause: unknown.

Other neurological syndromes

Management • Exclude drug causes—common culprits:β-blockers, H2 antagonists, neuroleptics, lithium, TCAs, anticonvulsants • Exclude peripheral neuropathy or ischaemic restpain • Iron deficiency (with or without anaemia) is associated in 1 in 3 sufferers so check FBC and serum ferritin • Also check:U&E, Cr, eGFR, fasting blood glucose, andTFTs • Try non-drug measures first—reassurance, information, walking/ stretching, warmth, relaxation exercises, massage • Drugs—dopamine agonists are often effective, e.g. ropinirole, pramipexole • Refer if severe symptoms or diagnosis is indoubt K.A. Ekbom (1907–77)—Swedish neurologist Patient support RLS-UK F 01634 260483 M www.rls-uk.org

Wernicke’s encephalopathy Thiamine deficiency causing nystag-

mus, ophthalmoplegia, and ataxia. Other eye signs, e.g. ptosis, abnormal pupillary reactions, and altered consciousness or confusion may also occur. Consider in any patient with symptoms and a history of alcoholism.

Management  Refer for confirmation of diagnosis. Meanwhile start thiamine 200–300mg od po to prevent irreversible Korsakoff’s syndrome. In severe cases admit as a medical emergency. K. Wernicke (1848–1904)—German psychiatrist.

Korsakoff’s syndrome  d ability to acquire new memories. May follow Wernicke’s encephalopathy and is due to thiamine deficiency. Confabulation to fill gaps in memory is a feature. Refer for specialist advice on management. S.S. Korsakoff (1853–1900)—Russian neuropsychiatrist.

Gilles de la Tourette syndrome  bp.911 Huntington’s disease (chorea)  Autosomal dominant trait. Testing can identify affected individuals before symptoms occur. Pre-conceptual and antenatal testing is available and should be offered to any couple with a family history on either the mother’s or the father’s side. Presents with movement abnormalities (e.g. hemichorea and rigidity) and dementia. Memory is relatively spared compared to cognition. Refer for expert advice. G. Huntington (1851–1916)—US physician. Friedreich’sataxia The most common inherited ataxia (autosomal recessive). Prevalence—1:50,000. Presents in adolescence with progressive gait and limb ataxia, loss of proprioception, pyramidal weakness, and dys­arthria. Extra-neurological involvement includes hypertrophic cardiomyopathy (most patients) and DM (10%). Treatment is supportive. Most patients become chairbound with in 15y and die in the 4th or 5th decade from cardiac or pulmonary complications. N.Friedreich (1825–82)— German neurologist. Patient support Ataxia UK F 0845 644 0606 M www.ataxia.org.uk

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Neurological rehabilitation problems New symptoms or limitations  Consider: • Is it due to an unrelated disease (e.g. change in bowel habit in someone who has had a stroke might indicate bowel cancer)? • Is it due to an incidental infection (e.g. UTI, chest infection)? • Is it due to a relapse (e.g. acute relapse in MS, TIA or further stroke in a stroke patient)? • Is it due to a side effect of treatment (e.g. acute confusion, involuntary movements or the on-off effect in a patient withPD)? • Is it part of a gradual progression (e.g. in MS, MND, brain tumour)? Treat any cause of deterioration identified. If no cause is found, consider re-referring for specialist review and/or referring to the multidisciplinary rehabilitation team involved with the patient. General principles of rehabilitation  bp.204 Fatigue  Consider and treat factors that might be responsible: • Depression • Disturbedsleep • Chronicpain • Poor nutrition Action  Review support, diet, and medication; encourage graded aerobic exercise; consider a trial of amantadine 200mg/d to improve symptomsN. Depression and anxiety  Common. Diagnosis can be difficult. Standardized questionnaires, e.g. PHQ-9 (b p.1001), are helpful for screening. Action  Give opportunities to talk about the impact of the illness on lifestyle. Jointly identify areas where positive changes could be made, e.g. referral to day care to widen social contact. Consider referral for counselling or to a self-help/support group. Consider antidepressant medication and/or referral to psychiatric services.

Emotionalism  If the patient cries (or laughs) with minimal provocation, consider emotionalism—impairment in the control of crying. Reassure. Sexual and personal relationships  Problems are common. Useful information sheets are available at M www.outsiders.org.uk Communication problems Speech therapy assessment is vital. Consider support via dysphasia groups and communication aids, e.g. simple pointing board (take advice from speech therapy andOT). Poorvision  Refer to an optician in the first instance. If corrected vision is still poor refer for ophthalmology review. Respiratory infections  Common. Treat with antibiotics unless in terminal stages of disease. Advise pneumococcal and influenza vaccination. Venous thromboembolism  Common but clinically apparent in 3 proven UTIs in 1y refer to specialist incontinence service or urology for further assessment • Incontinence b pp. 450–3 • Nocturia Desmopressin 100–400 micrograms po or 10–40 micrograms intranasally may be helpful • Urgency Modify environment, e.g. provide commode; try anticholinergic, e.g. tolterodine 2mg bd or oxybutinin 5mg tds. If not settling refer for specialist assessment

Bowel problems • Dysphagia Common. Fluids are more difficult to swallow than semisolids. Formal assessment by trained staff is essential. Feeding through NG tube or percutaneous endoscopic gastrostomy (PEG) may be needed long- or short-term—in terminal disease (e.g. MND), weigh provision of nutrition against prolongation of poor-qualitylife • Constipation Difficulty with defecation or BO 30min prior to emollients once or twice daily. Quantities to prescribe—see Table17.2 • Creams are suitable for moist or weeping lesions, and ointments for dry, lichenified, or scaly lesions or where a more occlusive effect is wanted. Lotions may be useful when minimal application to a large or hair-bearing area is needed or for the treatment of exudative lesions • Inclusion of urea or salicylic acid i penetration of the corticosteroid Cautions and contraindications  Topical steroids: • Are of no value in the treatment of urticaria • Are contraindicated for rosacea and not recommended foracne • May worsen ulcerated or secondarily infected lesions • Should not be used indiscriminately for pruritus—they will only be of benefit if inflammation is causing theitch • Should not be used long-term (>7–14d) on the face (and keep away from eyes) or for children. Use potent or very potent corticosteroids on the face only under specialist supervision 0 For perioral inflammatory lesions use hydrocortisone 1% for ≤7d or, if infected (e.g. angular cheilitis), hydrocortisone + miconazolecream. • Potent topical or systemic steroids used to treat patients with ­psoriasis can result in rebound relapse, development of generalized ­pustular psoriasis, and/or local and systemic toxicity.

Treatment of skin conditions

Table17.2  Quantities of emollients and corticosteroids to prescribe Area affected

Emollient* Cream/ointment Lotion

Topical steroid**

Face/neck

10–30g

100mL

15–30g

Both hands

25–50g

200mL

15–30g

Scalp

50–100g

200mL

15–30g

Both arms

100–200g

200mL

30–60g

Both legs

100–200g

200mL

100g

Trunk

400g

500mL

100g

Groins/genitalia

15–25g

100mL

15–30g

  Amounts are for an adult for 2x/d application for1wk. **   Amounts are for an adult for once daily application for2wk. *

0 One fingertip unit (distance from the tip of the adult index finger to the first crease) of steroid cream is sufficient to cover an area 2x the size of the flat adultpalm.

Box17.1  Ingredients that may cause skin sensitization Beeswax Benzyl alcohol Butylated hydroxyanisole Butylated hydroxytoluene Cetostearyl alcohol (including acetyl and stearyl alcohol) Chlorocresol Edetic acid(EDTA) Ethylenediamine Fragrances Hydroxybenzoates (parabens)

Imidurea Isopropyl palmitate N-(3-chloroallyl)hexaminium chloride (quaternium15) Polysorbates Propyleneglycol Sodium metabisulphite Sorbicacid Wool fat/related substances, including lanolin

Table17.3  Topical corticosteroid preparation potencies Potency

Examples

Mild

Hydrocortisone 0.1–2.5%, Dioderm®, Mildison® •  With antimicrobials Canesten® HC, Fucidin® H, Timodine® •  With crotamiton Eurax-Hydrocortisone®

Moderate

Betnovate®-RD, Eumovate®, Synalar® 1 in 4 dilution •  With antimicrobials Trimovate® •  With urea Alphaderm®, Calmurid® HC

Potent

Very potent

Betamethasone valerate 0.1%, Betacap®, Elocon®, Locoid®, Synalar®

• With antimicrobials Aureocort®, Betnovate®-C or -N, Fucibet® •  With salicylic acid Diprosalic® Dermovate®, Nerisone®Forte •  With antimicrobials Dermovate®-NN

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Changes in skin colour and eruptions Pallor Non-specific sign which may be racial, familial, or cosmetic. Pathology suggested includes anaemia, shock, Stokes–Adams attack, vaso­ vagal faint, myxoedema, hypopituitarism, and albinism. Erythema  bp.596   Hypo- and hyperpigmentation  bp.600 Linear lesions  Consider: • Koebner phenomenon (lesions arise in area of injury, e.g. in scratches)—occurs in psoriasis, eczema, lichenplanus • Linear urticaria • Self-inflicted trauma—dermatitis artefacta • Reaction to garden plants—psoralen-induced phytophotodermatitis • Impetigo—may spread along scratchmarks • Herpes zoster—at the edge of a dermatome • Stasis dermatitis—may follow varicoseveins

Ring-shaped (annular) lesions  Consider: • Granuloma annulare • Pityriasisrosea • Psoriasis • Erythema multiforme • Lichenplanus • Discoideczema • Basal cell carcinoma • Orf • Urticaria • Fungal infection, e.g. ringworm • Erythema migrans associated with Lyme disease • Burns (especially on a child—may be non-accidental injury) • Cutaneous T-cell lymphoma(rare) White patches  Consider all causes of patchy hypopigmentation:

• Vitiligo • After inflammation—cryotherapy, eczema, psoriasis, morphoea • Pityriasis alba—white post-inflammatory patch on a child’s face. No treatment needed • Exposure to some chemicals—substituted phenols, hydroquinone • Certain infections—pityriasis versicolor • Tuberous sclerosis • Halo naevus (pale area around amole) • Piebaldism (from birth—associated with a white forelock) • Extensive hyperpigmentation, e.g. chloasma—patches of normal skin may appear hypopigmented

White spots  Consider: • Pustules/whiteheads, e.g. due to acne, folliculitis, or rosacea • Molluscum contagiosum—white spots with a pearl-like appearance • Milia—small white spots usually on upper arms/face of children; resolve spontaneously Brown spots  Consider: • Melanoma • Freckles • Café-au-lait spots— • Moles >5 associated with • Lentigos—like neurofibromatosis freckles but darker and not affected by • Basal cell carcinoma • Seborrhoeicwarts sunlight

• Senile keratoses • Dermatofibroma • Systemic disease • Addison’s disease • Acanthosis nigrans • Haemochromatosis

Changes in skin colour and eruptions

Scaling • Silvery scaling on the surface of red patches Psoriasis • Fine scaling accompanied with rash Pityriasis • Coarse, scaly skin with no rash Ichthyosis • Localized, not itchy Bowen’s disease

Yellow crusting  Usually due to staphylococcal infection (impetigo). Telangiectasia  Dilated distal venule/arteriole (spider naevus). Causes: • Congenital—e.g. hereditary haemorrhagic telangiectasia • Venous disease in the leg—e.g. venousstars • Rosacea—facial • Excess oestrogen—e.g. liver disease; the COC pill; pregnancy • Skin atrophy—e.g. ageing skin, radiation dermatitis, topical steroids • AroundBCC

Spider naevi  Small red lesions (barely visible—0.5cm diameter) in

superior vena cava distribution, i.e. on the arms, neck, and chest wall. Large arteriole with numerous small vessels radiating from it giving the appearance of a spider—hence the name. Pressure applied to the central arteriole (e.g. with a pointed object) causes blanching of the whole lesion. >2 spider naevi is abnormal. Causes: • Cirrhosis—most frequently, alcoholic • Oestrogen excess—usually in association with chronic liver disease • Rheumatoid arthritis—rarely • Viral hepatitis (transient) • Pregnancy—usually disappear in the final trimester

Blisters  bp.594 Subcutaneous nodules Consider:

• Tumour • Cysts • Rheumatoid arthritis • Neurofibroma • Granuloma annulare • Xanthelasma

• Furuncle • Sarcoid • Polyarteritis

Purpura  Blue-brown discoloration of the skin due to bleeding within it.

Petechiae are small dot-like purpura whilst ecchymoses are more extensive. Treat the cause. Causes: • Idiopathic, e.g. idiopathic pigmented purpura (brownish punctate lesions on thelegs) • Vessel wall defects Vasculitis, paraproteinaemia, infection (e.g. meningococcal meningitis, septicaemia, glandular fever), i intravascular pressure (e.g. venous disease) • Clotting defects Abnormal platelet function; thrombocytopenia; anticoagulant therapy; coagulation factor deficiency • Defective dermal support Dermal atrophy (e.g. ageing, steroids, disease); scurvy (vitamin C deficiency) • Referral of patients with purpura • Admit unwell patients with new purpura/petechiae as an emergency • Refer well children/young adults with unexplained petechiae immediately to be seen the samedayN • For well older adults with unexplained bruising, bleeding, or purpura, check FBC, blood film, clotting screen, and ESR/viscosity/CRPN

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Itching and blistering of theskin Itching  In any patient presenting with pruritus or itch, ask:Are there skin lesions present? Skin lesions present Search for unexcoriated lesions. Investigations are not usually needed. Exceptions are patch testing for contact dermatitis and skin biopsy for dermatitis herpetiformis. Causes: • Infections—viral, e.g. • Urticaria chickenpox;fungal • Contact dermatitis and allergies • Dermatitis herpetiformis to food anddrugs • Lichenplanus • Pricklyheat • Senile atrophy • Skin infestations, e.g. scabies, • Psychological causes* pediculosis, insectbites *  excessive excoriation causes lichenification of theskin.

Skin lesions absent  Large differential diagnosis. Look for pallor, jaundice, weight d, LN enlargement, and abdominal organomegaly. Investigate as necessary—consider urinalysis (dipstick and MSU), FBC, ESR/CRP, serum ferritin, LFTs (including alkaline phosphatase), U&E, Cr and eGFR, glucose, serum Ca2+, TFTs, and CXR. If still undiagnosed—refer. Causes: • Hepatic—obstructive jaundice, pregnancy • Endocrine—DM, thyrotoxicosis, hypothyroidism, hyperparathyroidism • Renal—chronic renal failure • Haematological—polycythaemia vera, iron deficiency, leukaemia, Hodgkin’s disease • Malignancy—any carcinoma •  Drug allergies • Psychological—obsessive states, schizophrenia • Rare causes—diabetes insipidus, roundworm infection

Blisters Result from separation of skin layers. Type of blister depends on level of cleavage of the skin—subcorneal or intraepidermal blisters rupture easily, subepidermal blisters are much tougher. Causes: • Subcorneal Pustular psoriasis (b p.619), bullous impetigo (b p.632) • Intraepidermal Eczema (b pp. 606–11), HSV (b p.634), VZ— chickenpox (b p.652) or shingles (b p.653), pemphigus, friction • Subepidermal Cold or heat injury (burns—b p.1114), pemphigoid, dermatitis herpetiformis, linear IgA disease • Other Insect bites (may cause cleavage at any level)

PemphigoidG  Autoimmune disorder. • Bullous pemphigoid Usually affects the elderly. An urticarial reaction may precede onset of blistering. Large, tense blisters arise on red or normal skin on the limbs, trunk, and flexures. Oral lesions in 20–30%. May be localized to one site, e.g. lower leg. Differential diagnosis: pemphigus, dermatitis herpetiformis, linear IgA disease • Cicatricial pemphigoid Mainly affects mucous membranes in the eyes/ mouth. Scarring results in visual loss. Refer to ophthalmology • Pemphigoid gestationis Rare but characteristic bullous eruption associated with pregnancy. Remits after delivery but often recurs in subsequent pregnancies. bp.805

Itching and blistering of theskin

Management  Refer to dermatology for skin biopsy and confirmation of diagnosis. Treatment is usually with oral steroids (prednisolone 30–60mg daily initially—reducing as symptoms improve). Other treatments include antibiotics and nicotinamide, azathioprine, or other immunosuppressants. Prognosis  Self-limiting in 50%—steroids are often stopped after72y.

PemphigusG Uncommon, autoimmune disorder affecting skin and mucous membranes. Affects adults (peak incidence 30–70y). Cause: 90% have detectable circulating autoantibodies. Associated with other auto­ immune disorders, e.g. myasthenia gravis. Presentation  50% present with oral lesions. Suspect in anyone presenting with mucocutaneous erosions/blisters. Flaccid superficial blisters then appear—sometimes months later—over scalp, face, back, chest, and flexures. As blisters are fragile they burst early and the condition may present as crusted erosions. Untreated the condition is progressive. Management  Refer to dermatology. Treatment is with high-dose systemic steroids or other immunosuppressive agents. Treatment is continued long-term although occasional remissions occur. Before treatment with steroids 75% of patients died in 5 (2:1). Peak incidence: third/fourth decade. Consists of itchy vesicular skin rash on elbows (extensor surface), knees, buttocks, and scalp, which are often broken by scratching to leave excoriations. Closely related to coeliac disease (b p.412); 2–5% of patients with coeliac disease have dermatitis herpetiformis but classic symptoms of coeliac disease are uncommon. Differential diagnosis: scabies, eczema, linear IgA disease. Management  Refer to dermatology for skin biopsy to confirm diagnosis. Responds to withdrawal of gluten—although may take up to 1y. Controlled in the interim with dapsone or sulfapyridine.

Epidermolysis bullosa  A group of genetically inherited diseases char-

acterized by blistering on minimal trauma. Range from being mild and trivial to being incompatible with life. The most common form is simple epidermolysis bullosa (autosomal dominant)—blistering is caused by friction, is mild and limited to hands and feet. Patients are advised to avoid trauma.

Linear IgA disease Rare condition of blisters and urticarial lesions on the back and extensor surfaces. Refer to dermatology. Responds to dapsone. Staphylococcal scalded skin syndrome  bp.903 Further information Electronic dermatology atlas M www.dermis.net British Association of Dermatologists M www.bad.org.uk • Guidelines for the management of pemphigus vulgaris(2003) • Guidelines for the management of bullous pemphigoid(2002)

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Erythema Erythema is redness of the skin—usually due to vasodilatation. It may be localized (e.g. pregnancy—on the palms), generalized (e.g. flushing) or take the form of a red rash (e.g. drug eruption, viral exanthem). • Erythroderma (exfoliative dermatitis) Erythema affecting >90% skin surface. Rare, but systemic effects of skin failure are potentially fatal. 4:5 82:1. Typical patient is middle-aged or elderly. Patchy erythema becomes universal in 4. Advise warm housing/clothing, gloves, and woolly socks. In severe cases oral nifedipine mayhelp.

Erythema

Erythema abigne  Reticulate pigmented erythema due to

heat-induced damage. Common in the elderly—especially from sitting in front of the fire or using hot water bottles to alleviate pain. In younger patients may be caused by laptop computers balanced on thighs. Explain the cause. Resolves spontaneously.

Viral rash Common, particularly in children. Appears suddenly (over

hours) and is associated with symptoms of the underlying viral infection. The rash may take many forms but is usually widespread, red, maculopapular and blanches on pressure. In most cases, the underlying virus cannot be identified, but viral infections with characteristic rashes include: • Fifth disease (slapped cheek) bp.652 • Chickenpox bp.652 • Roseola infantum bp.652 • Measles bp.652 • Hand, foot, and mouth bp.652 • Rubella bp.652 Management  In all cases, no specific treatment is needed for the rash— treat the underlying viral infection symptomatically. Rash illness in pregnancy  bp.804

Lyme disease  Cause:Borrelia burgdorferi. Spread: transmitted by ticks— usually from deer or sheep. Presentswith: • Erythema migrans (75%—see Figure17.5)—a red macule/papule on the upper arm, leg, or trunk 7–10d after a tick bite, which expands over days/weeks to form a ring with central clearing; diameter can be up to 50cm; further smaller lesions then develop elsewhere • Flu-like illness • Lymphadenopathy ± splenomegaly • Arthralgia

Symptoms are typically intermittent and changing. Complications include neurological abnormalities, aseptic meningitis, myocarditis, and arthritis. Management  Confirm diagnosis with serology. Treatment is usually with 2–3wk course of doxycycline—take microbiology advice. X Treatment with antibiotics after a tick bite but before symptoms have arisen is controversial. Removal of ticks  b p.1108

Figure17.5  Erythema migrans following a tickbite

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Pigmentation disorders Hypopigmentation  Lack of skin pigmentation. Maybe: • Generalized Albinism; phenylketonuria; hypopituitarism • Patchy Vitiligo; tuberous sclerosis; morphoea; pityriasis alba; or after inflammation (e.g. post-cryotherapy, s to eczema or psoriasis), infection (e.g. pityriasis versicolor), or exposure to chemicals (substituted phenols, hydroquinone) • Around a mole—halonaevus Hyperpigmentation  Excess skin pigmentation. Maybe: • Genetic Racial; freckles; neurofibromatosis; Peutz–Jegher’s syndrome • Due to drugs Amiodarone (blue-grey pigmentation of sun-exposed areas); psoralens; minocycline (blue-black pigmentation in scars and buccal mucosa); chloroquine (blue-grey pigmentation of face and arms); chlorpromazine (grey pigment in sun-exposed sites); cytotoxics • Endocrine Addison’s disease; chloasma; Cushing’s syndrome • Nutritional Ingestion of carrots (carotinaemia); malabsorption • Post-inflammatory Varicose eczema; lichen planus; systemic sclerosis • Other Benign naevi; malignant melanoma; chronic renal failure (lemon yellow); liver disease (jaundice); acanthosis nigricans Freckles and lentigines • Freckles are small, light brown macules—typically facial—which darken in the sun. They are common particularly in red heads and develop in childhood. Require no treatment • Lentigines Are also brown macules but more scattered and do not darken in the sun. Most common in elderly sun-exposedskin

Chloasma Patterned macular symmetrical facial pigmentation, usually involving the forehead and/or cheeks (see Figure17.6), 5 >> 4. Peak age 20–40y. Affects dark skins > fairskins. Risk factors  Pregnancy (usually fades after delivery); taking CHC or depot contraceptives (may be slow to fade when stopped); use of cosmetics, perfumes, or deodorantsoap. Management  Reassurance is normally all that is needed. Consider: • Stopping hormonal contraception • Avoid irritating the skin with strong soaps/abrasive cleaners • Vigorous photoprotection using sunscreen andhat • 20% azelaic acid cream or 0.05% tretinoin cream may be used but requires application for >6mo to have any effect • Rarely, patients are so worried about their appearance that camouflage cosmetics are warranted • Laser resurfacing—mixed results—refer for expertadvice

Albinism  Prevalence: 1:20,000. Rare genetic syndrome (autosomal

recessive inheritance) in which the melanocytes are unable to produce skin, hair, or eye pigment. Patients have white hair, pale skin, pink eyes, poor sight, photophobia, and nystagmus. Several different varietiesexist. Management  Strict sun avoidance, sunglasses, sunscreens, refer any skin lesions for biopsy (i risk squamous cell carcinoma).

Pigmentation disorders

Figure17.6  Chloasma of thecheek

Figure17.7  Vitiligo on the forearms

Reproduced with permission from M www.dermnetnz.org

Reproduced with permission from M www.dermnetnz.org

Vitiligo  Affects 1% of the population. 4 = 5. Peak age of onset: 10–30y. Cause: autoimmune; 18% have a family history. Associations: pernicious anaemia, Addison’s and thyroid disease. Presentation  May be precipitated by injury or sunburn. Presents as smooth sharply defined white macules or patches which contain no melanocytes (see Figure17.7). Skin appears bright white under Wood’s light. Often symmetrical distribution. Hair may also be affected. Most common sites: hands, wrists, knees, neck, face, around eyes, andmouth. Differential diagnosis Post-inflammatory hypopigmentation, chemical exposure. Management  Prognosis is variable—some develop a few lesions which remain static; some progress to larger depigmented areas; some repigment. There is no cure. Advise use of sunscreens for affected areas (ACBS prescription); camouflage cosmetics (refer to Changing Faces for advice on application, ACBS prescription). Consider referral to dermatology for consideration of topical steroid treatment, topical calcineurin inhibitors (tacrolimus, pimecrolimus—unlicensed), or phototherapy. Morphoea (localized scleroderma)  5:4 83:1. Cutaneous localized form of scleroderma. Pathologically distinct from lesions of systemic sclerosis. Internal disease is not associated. Cause: autoimmune but not fully understood—may follow trauma. Presents with round/oval plaques of induration and erythema which become smooth, shiny and white, with violet borders. Eventually leaving atrophic hairless pigmented patches. Affects trunk/proximal limbs. No established treatment—topical steroids are often tried. Usually resolves spontaneously in3–5y. Further information British Association of Dermatologists Guidelines for the management of vitiligo (2008) M www.bad.org.uk

Patient support  Changing Faces Skin Camouflage Service Mwww.changingfaces.org.uk

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Hair and sweat gland problems Hair loss or alopecia May be diffuse or localized, scarring or nonscarring. Treatment is according to cause. Differential diagnosis: • Diffuse non-scarring Male pattern baldness (responds to topical minoxidil and 1mg finasteride orally but hair loss returns as soon as stopped—neither treatment is available on NHS prescription); hypothyroidism; iron deficiency; malnutrition; hypopituitarism; hypoadrenalism; drug-induced • Localized non-scarring Alopecia areata; ringworm; traumatic; hair pulling; traction; SLE; secondary syphilis • Scarring Burns; radiation; shingles; tertiary syphilis; lupus erythematosus; morphoea; lichen planus Alopecia areataG  Common chronic inflammatory disease affecting the hair follicles ± nails (710%). Patches of hair loss usually on the scalp (see Figure17.8) but can affect any hair-bearing skin. 20% have a family history. Consider alternative diagnosis of tinea capitis if scales/erythema present. Management  Investigation is usually unnecessary. If mild hair loss, reassure and monitor hair loss; refer more severe cases to dermatology. Treatment options include topical/locally injected/systemic steroids ± contact immunotherapy. 740% recover in other finger nails—due to habitual rubbing/ picking at the cuticle) Chronic paronychia

Longitudinal

Lichen planus Darier’s disease (keratosis follicularis— genetic disorder appearing in adolescence mainly affecting skin/nails)

Nail fold telangiectasia

Dilated capillaries and erythema at the nail fold

Connective tissue disorders

Tumours of the nail fold

Benign

Viral warts Myxoid (mucus) cysts (treat with steroid injection or cryotherapy) Periungual fibroma (associated with tuberous sclerosis—appear at puberty)

Malignant

Melanoma Squamous cell carcinoma

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Atopiceczema Affects 15–20% of schoolchildren and 2–10% of adults—usually starts ½ are free of eczema by18mo. Children >18mo Involves antecubital and popliteal fossae (see Figure17.10), neck, wrists, and ankles. Lichenification, excoriation, and dry skin are common. Face may be erythematous and have typical infraorbital folds. Loss of self-esteem, behaviour, and sleep problems are common. Adults  Most commonly irritant hand dermatitis (see Figure17.11) in a person with past history of atopic eczema—b p.609. Afew continue to have generalized atopic eczema; 20y. May interfere with employment/social activities. Exacerbated by stress. Diagnosis  Itchy skin plus≥3of: • Itching in skin creases • Generally dryskin • History of asthma or hayfever • Visible flexuraleczema • Onset in the first 2y oflife Assessment  Askabout: • Family (two-thirds) and personal history of atopy andeczema • Onset and distribution of the disease • Aggravating factors (pets, irritants, e.g. soaps/detergents, allergens) • Sleep disturbance due to itching/rubbing • Impact on quality of life (school work, career, sociallife) • Previous treatments (including dietary restrictions), expectations of treatment and other medications being taken (e.g. steroids for asthma) • Nipple eczema May be Paget’s disease of the breast (b p.688). Refer urgently to a breast surgeon if no response to topical treatmentN.

Complications • Skin thickening and scaling • Bacterial infection Secondary infection (usually with Staph. aureus) commonly causes exacerbations and may not be seen as obvious infection. Bacterial infection is suggested by presence of crusting or weeping or sudden deterioration of eczema • Viral infection i susceptibility to infection, e.g. viral warts, molluscum. Eczema herpeticum—propensity to develop widespread lesions with HSV and VZ; may require admission and IV aciclovir • Cataracts Rarely occur in young adults with very severeeczema • Growth retardation Children with severe eczema, cause unknown. Agrowth chart should be kept for children with chronic severeeczema

Atopiceczema

Figure17.9  Infantile eczema on thecheeks

Figure17.10  Childhood eczema involving both poplitealfossae

Figure17.11  Hand (contact) dermatitis in anadult Figures17.9–17.11 reproduced with permission from New Zealand Dermatological Society Incorporated. Published online at M www.dermnetnz.org

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Management • Education is time well spent—explain the condition and provide verbal and written information on a stepped approach to care and management of flaresN • Advise—loose cotton clothing; avoid wool (exacerbates eczema); avoid excessive heat; keep nails short; gloves inbed • If a specific irritant is identified (e.g. house dust mite, pets) thenavoid

Specific treatment • Emollients Topical creams/ointments and bath emollients—use regularly on skin and as soap substitutes, even if skin is clear. May need to try several to find one that suits. Ideally apply 3–4x/d to moist skin. Ensure enough is supplied. Addition of an antipruritic, e.g. lauromacrogol to the emollient, may help break the scratch–itch cycle. Addition of an antiseptic to bath emollient may d bacterial infection • Topical steroids Prescribe the least potent strength that is effective. Use od or bd. Ointments are preferable on dry, scaly eczema; creams on wet, exudative eczema. Emollients d steroid requirement • Antibiotics For infected eczema—topical (alone or in combination with steroid, e.g. Fucidin® H) or oral (e.g. flucloxacillin or erythromycin 250–500mg qds for 2wk). Swab if antibiotic treatment is ineffective • Oral steroids Rescue therapy while waiting for an urgent consultant opinion. Only use short courses, e.g prednisolone 20–30mg od for5d • Topical immunosuppressants, e.g. tacrolimus—on consultant adviceonly • Antihistamines Sedative antihistamines given nocte d desire to itch, e.g. promethazine, hydroxyzine • Bandages Excoriated or lichenified eczema—ichthammol or zinc and calamine. Bandages can be applied at night on top of steroid ointment. Refer to dermatology • Wet wrapping Used for exudative eczema—tubigrip bandage or tubular gauze soaked in emollient is applied and covered with a dry bandage. Refer to dermatology • Dietary manipulation Few ( 5. Unknowncause • Presentation Intensely itchy, coin-shaped lesions on limbs. Tend to be symmetrical. May be vesicular or chronic and lichenified • Differential diagnosis Tinea corporis; contact dermatitis, psoriasis • Management Often clears spontaneously after a few weeks but tends to recur. If treatment is needed, use a moderate or potent topical steroid. Secondary infection is common—treat with topical/systemic antibiotics. Asedating antihistamine (e.g. hydroxyzine 50mg nocte) may be useful if sleep is disturbed by itching

Venous (stasis, varicose)eczema • Middle-aged/elderly patients.5>4 • Associated with underlying venous disease • Early signs Capillary veins and haemosiderin deposition around the ankles and over prominent varicoseveins • Later signs (see Figure17.12) Eczema ± lipodermatosclerosis (fibrosis of the dermis and subcutaneous tissue) ± ulceration • Management Emollients ± mild or moderate steroid ointment (avoid long-term use) and compression hosiery. Treat venous disease (bp.288) or ulceration (b p.612) on its own merits

Figure17.12  Varicose eczema showing haemosiderin deposition, excoriated ecze­ma­ tous lesions, and lipodermatosclerosis

Reproduced with permission from New Zealand Dermatological Society Incorporated. Published online at:M www.dermnetnz.org

Asteatotic eczema (eczema craquelé) •  Risk factorsi age; overwashing; dry climate; hypothyroidism; diuretics • Presentation Dry itchy eczema with fine, crazy-paving pattern of fissuring and cracking of the skin of thelimbs • Management Treat with emollients—occasionally a mild topical steroid is required

Other eczemas

Pompholyx • Sago-like intensely itchy vesicles on the sides of fingers ± palms/soles • No associated atopic eczema or contact dermatitis • Young adults. More common in warm weather. Frequently recurrent • Treat with emollients and topical steroids (some need potent steroids) • Treat any infection with oral antibiotics • In severe cases, refer to dermatology for wet dressings

Lichen simplex chronicus Area of lichenified eczema due to repeated rubbing/scratching. May be due to habit or stress. Treatment: take scrapings to exclude fungal infection; topical steroids; occlusive dressing (e.g. Duoderm®) to protect skin from scratching.

Seborrhoeic dermatitis  Chronic scaly eruption affecting scalp, face and/or chest. Differential diagnosis: psoriasis, rosacea, contact dermatitis, fungal infection. Five patterns: • Scalp and facial involvement Most common in young men. Excessive dandruff, itchy scaly erythematous eruption affecting sides of the nose, eyes, ears, hairline. May be associated blepharitis • Petaloid Dry, scaly eczema over the pre-sternalarea • Pityrosporum folliculitis Erythematous follicular eruption with papules/pustules over theback • Flexural Most common in the elderly. Axillae, groins, and submammary areas. Moist intertrigo. Associated with s candida infection • Infantile bp.903 Treatment • Facial, truncal, and flexural involvement Imidazole + hydrocortisone. Pityrosporum folliculitis may respond to itraconazole 200mg od for 7d or fluconazole 50mg od for2wk • Scalp lesions Ketoconazole or coal tar shampoo. In resistant cases, apply 2% sulphur + 2% salicylic acid cream several hours before shampooing • Recurrence requiring repeated treatment is common. Consider maintenance treatment with topical antifungal every otherweek 0 Severe or recalcitrant seborrhoeic dermatitis is an indicator disease for HIV infection—offer HIV testing.

Dandruff  Is exaggerated physiological exfoliation of fine scales from an

otherwise normal scalp. More severe forms merge with seborrhoeic dermatitis and treatment is thesame.

Further information CKS Seborrhoeic dermatitis (2008) M www.cks.nhs.uk British HIV Association, BASHH and British Infection Society UK national guidelines for HIV testing (2008) M www.bashh.org

Patient information and support

National Eczema Society F 0800 089 1122 M www.eczema.org

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Ulcers Leg ulcer  Painful and debilitating condition, affecting 1% of the adult population and 3.6% of those>65y. Cause  >90% result from arterial disease, venous disease, or neuropathy. Other causes: trauma, obesity, immobility, vasculitis (rheumatoid arthritis, SLE, PAN), malignancy, osteomyelitis, blood dyscrasias, lymphoedema, self-inflicted. Commonsites • Arterial Shin, toes, over pressure points (under heel, over malleoli) • Venous Above medial or lateral malleoli of theankle • Neuropathic Sole of foot, over pressure points History  Askabout: • Duration of ulceration • Pain—painful, unless neuropathic when often painless • Mobility • Past history of ulceration, DVT, or varicose vein surgery • History of trauma to thelimb • Systemic disease—e.g. DM, peripheral vascular disease, RA Examination • Ulcer Position; evidence of infection; surrounding callus—typical of neuropathic ulcers; evidence of tracking to involve the bones of thefoot • Leg Pulses; varicose veins and/or signs of venous hypertension— haemosiderin pigmentation, varicose eczema, atrophie blanche (white lacy scars), lipodermatosclerosis; sensation—d when peripheral neuropathy; range of joint movement Investigation • Bloods FBC, ESR, VDRL, blood glucose • Ankle–brachial pressure index (b p.237) • Swab for M,C&S if any signs of cellulitis/infection • Diabetic ulcer—if signs of infection, X-ray foot to exclude osteomyelitis Management • Arterial ulcers Refer to vascular surgery • Diabetic foot ulcers Refer to a specialist diabetic footteam • Venous ulcers If ABPI >0.8, can be managed in the community with graduated compression bandaging (elastic bandages applied in multiple layers over a non-adherent dressing). Change dressings 1–2x/wk. Keep skin under the bandage moist with simple emollients and treat surrounding eczema with topical steroids. Give analgesia. Encourage walking, weight d if obese, and elevation of the leg when resting. 65–70% heal in 1cm diameter. Large ones have i risk of malignancy • Junctional Flat, round/oval, brown/black, 2–10mm diameter. Common sites:soles, palms, genitalia • Intradermal Dome-shaped papule/nodule commonly on the face or neck. May be pigmented • Compound 4; young adult > elderly) and usually asymptomatic. Firm (sometimes pigmented) nodule 5–10mm in diameter, that may occur following an insect bite or minor trauma (see Figure17.25). Most common site: lower legs. Treatment is with excision biopsy of symptomatic or diagnostically doubtful lesions. Keratoacanthoma Rapidly growing nodular tumour ( 4. Most common site: lower leg in 5 (50%); back in 4. Macular lesion with variable pigmentation (see Figure17.29a) • Nodular (see Figure17.29b) 20% UK cases. 4 > 5. Most common on trunk. Pigmented nodule grows rapidly and may ulcerate • Lentigo Alentigo maligna arises in sun-damaged skin—usually on the face—and melanoma develops many years afterwards within it. Most common >60y—especially if outdoor occupation • Acral lentiginous 35–60% melanoma in black-skinned populations. Affects palms, soles, and nail beds. Often detected late. Poor prognosis Risk factors  Sun exposure/sunbed use; genetic (10% have family history); multiple benign moles (>50 of >2mm diameter); congenital naevus; previous malignant melanoma; immunosuppression; fair skin type (red hair, blue eyes, and burns easily); severe sunburn in childhood/adolescence; 30% arise out of pre-existing moles, but risk of change in a benign mole (except dysplastic or congenital naevus) issmall. Assessment  Encourage patients to report changes in moles early. Check the ABCDEF criteria: • A Asymmetry of outline • C Colour variation • B Border irregularity • D Diameter • E Evolution—changes in size, shape, colour, and/or elevation • F Funny-looking’ mole—‘ugly duckling’ moles that stand out from the others are very discriminatory for nodular melanoma Management  Use the 7-point checklist (see Box 17.2) ± dermoscopy findings (if available) to identify changes needing referral. Refer all suspicious lesions for urgent dermatology assessment ± wide excision. Dermoscopy  0 Requires specialized training. Useful for distinguishing benign/malignant pigmented lesions. Use the 3-point checklist—see Box17.3. Specialist treatment  Best chance of cure comes with complete excision. Chemotherapy/radiotherapy are of little benefit, but biological therapies (e.g. interferon, interleukin-2, ipilimumab, vemurafenib) are commonly used. Atreatment vaccine is currently undergoing clinical trials. Prognosis  Relates to tumour depth at presentation. 5y survival:4mm deep—45%; metastases—10%.

Skincancer

(a)

(b)

Figure17.29  Malignant melanoma (a)superficial spreading (b)nodular

Box17.2  The 7-point checklist formoles

Score 2 points for any major feature and 1 point for any minor feature. Lesions scoring ≥3 points are suspicious—refer. Majorsigns  • Change in size—increase insize • Irregular shape—irregular • Irregularcolour border, asymmetry, elevation Minorsigns  • Change in sensation— • ≥7mm diameter including symptoms of minor • Inflammation irritation oritch • Oozing—including crusting/bleeding 0 One feature is enough to prompt referral if high level of suspicion. For low-suspicion lesions, monitor for change over8wk.

Box17.3  The 3-point dermoscopy checklist Each item scores 1 point—any lesion scoring 3 points warrants referral (sensitivity96%). • Asymmetry Of colour/structure (not shape) in ≥1 perpendicularaxis • Atypical pigment network Irregular holes and thick lines (broadened network); streaming (irregular finger-like projections at the edge of the lesion) and pseudopods (bulbous areas of pigmentation joined to the tumour body or pigmented network at the periphery of the lesion) are also atypical • Blue-white structures Blue-white veil (irregular, structureless area of confluent blue pigmentation with an overlying white ‘ground-glass’ haze) ± regression structures (e.g. scar-like depigmentation)

Further information Soyer P, Angenziano G, Zalaudek I,etal. Three-point checklist of dermoscopy. Dermatology (2004) 208:27–31 British Association of Dermatologists UK guidelines for the management of cutaneous melanoma (2010) M www.bad.org.uk NICE Improving outcome for people with skin tumours including melanoma:The Manual (2006) M www.nice.org.uk

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Squamous cell carcinoma (SCC) 20% of skin cancer in the UK.

Most common >55y; 4 > 5. May metastasize (10%). Usually develops in sun-exposed sites, e.g. face, neck, hands. May start within an actinic (solar) keratosis (b p.624) or de novo as a nodule which progresses to ulcerate and crust (see Figure17.30). Causes  Chronic sun damage, X-ray exposure, chronic ulceration and scarring (aggressive SCC may develop at the edge of chronic ulcers), smoking pipes and cigars (lip lesions), industrial carcinogens (tars, oils), wart virus, immunosuppression, genetic. Management  Refer urgently to dermatology. Treated with surgical excision ± LN biopsy. Large lesions may require skin grafting. Radiotherapy is an alternative for large lesions in elderly patients. Bowen’s disease Intraepidermal SCC. Common—typically occurs on the lower leg in elderly women. Lesions are flat-edged, pink/slightly pigmented scaly plaques (75% of skin cancer in the UK. Locally invasive but rarely metastasizes. Tends to occur in middle-aged/elderly patients, may be multiple and appears mainly on light-exposed areas—most commonly the face. 3 major types (see Figure17.32—all can be pigmented): • Nodular Most common. Starts as small pearly nodule ± surface telangiectasia. May necrose centrally leaving a small crusted ulcer with pearly, rollededge • Superficial ≥1 scaly erythematous plaques with pearlyedges • Morphoeic Waxy indurated plaque resemblingascar Dermoscopy of pigmented BCC  0 Requires specialized training: • Absence of pigment network • Specks of brown/grey pigment • Multiple blue-grey globules • Focal ulceration • Large blue-grey ovoid nests or blotches • Linear and arborizing (tree branch-like) telangiectasia • Structureless or leaf-like areas towards the edge of thelesion • Spoke wheel areas (like spokes of a wheel radiating from a centralhub) Causes  Sun exposure, X-ray irradiation, chronic scarring, genetic predisposition, arsenic ingestion Management  Complete excision is the ideal. Refer routinely—if low risk to GPwSI working in a community skin cancer clinic; if uncertain or any high-risk features to dermatology. High-risk features: • Site—nose/paranasal folds, scalp/temples, lips • Size>2cm • Immunosuppression • Previously treatedlesion • Genetic disorder associated with BCC, e.g. Gorlin’s syndrome Prognosis  Recurrence rate is 5% at 5y for all modalities of treatment. Development of new basal cell carcinoma at other sites is common.

Skincancer

Figure17.31  Bowen’s disease

Figure17.30  Squamous cell cancer showing ulceratednodule

(a)

(b)

(c)

Figure17.32  Basal cell cancer. (a)Nodular BCC—pearly nodule showing surface telangiectasia. (b)Superficial BCC—scaly erythematous patch with pearly, rolled edges. (c)Morphoeic BCC (can be difficult to see)—waxy induratedplaque

Further information British Association of Dermatologists M www.bad.org.uk • Multiprofessional guidelines for the management of the patient with primary cutaneous squamous cell carcinoma(2009) • Guidelines for management of Bowen’s disease(2006) • Guidelines for the management of BCC(2008) NICE Improving outcomes for people with skin tumours, including melanoma:The Manual (2006) M www.nice.org.uk

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Bacterial skin infection Impetigo  Superficial skin infection due to Staph. aureus.

Very common in childhood. Athin-walled blister ruptures easily to leave a yellow crusted lesion. May occur anywhere but most common on the face. Differential diagnosis: HSV, fungal infection (e.g. ringworm). Lesions spread rapidly and are contagious. Avoid spreading to other children—no sharing of towels, face flannels, etc.; some schools/nurseries/childminders prohibit attendance until lesions are cleared. Reassure that non-scarring: • Localized Treat with topical antibiotics (e.g. fusidic acidcream) • Widespread Treat with oral flucloxacillin or clarithromycin

Erysipelas and cellulitis Acute infection of the dermis. Often pre-

ceded by fever/‘flu-like’ symptoms. Usually affects face/lower leg. Appears as a painful, tender reddened area with a well-defined edge. Often the area is swollen and may blister. May be an obvious entry wound. Differential diagnosis: angio-oedema, contact dermatitis,gout. Management  • Severe infection Admit for IV antibiotics • If systemically well Mark the area before starting flucloxacillin 500mg qds or clarithromycin 500mg bd for 7–14d. Advise to seek help if infection is spreading or becoming systemicallyunwell • Facial infection Treat with penicillin V 500mg qds (flucloxacillin 500mg qds if staphylococcal infection suspected or clarithromycin 500mg bd if penicillin-allergic). Have a low threshold for admission • Recurrent infections (>2 episodes at one site). May need prophylactic long-term penicillin (e.g. penicillin V 250mg od or bd) with attention to skin care and management of any lymphoedema

Boils and carbuncles • Boil (furuncle) Acute infection of a hair follicle, usually with Staph. aureus. Ahard, tender, red nodule surrounding a hair follicle becomes larger and fluctuant after several days. Occasionally associated with fever ± malaise. Later may discharge pus and a central ‘core’ before healing; may leave a scar. Predisposing factors: usually absent—DM; HIV; obesity; blood dyscrasias; immunosuppressivedrugs • Carbuncle Swollen, painful area discharging pus from several points. Occurs when a group of hair follicles become deeply infected, usually with Staph. aureus. May be associated with fever ± malaise. Predisposing factors: malnutrition, cardiac failure, drug addiction, severe generalized dermatosis, prolonged steroid therapy,DM Management • Non-fluctuant lesions Apply moist heat to relieve discomfort, help localize the infection, and promote drainage • If fever or surrounding cellulitis or lesion is on the face Treat with oral antibiotics, e.g. flucloxacillin 500mg qds for 7d—clarithromycin 500mg bd is an alternative if allergic to penicillin • If large but localized, painful, and fluctuant Consider incision and drainage (0 do not attempt if you are not confident). Admission may be needed if young or uncooperative child, or the boil is in a sensitive

Bacterial skin infection

area, e.g. genital region, face, neck, axilla, breast. Afterwards treat with oral antibiotics until inflammation resolves • Admit If not settling with primary care treatment • If recurrent or chronic Take swabs for culture from lesions and carrier sites (nose, axilla, and groin); treat carrier sites with topical antibiotic (e.g. Naseptin® qds for 10d). Advise improved hygiene and use of antiseptics in the bath (e.g. chlorhexidine); consider long-term antibiotics (e.g. clarithromycin 500mgod)

Folliculitis Superficial infection of the hair follicles usually caused by Staph. aureus. Presents as pustules in hair-bearing areas, e.g. legs, beard area. Risk factors: obesity, DM, occlusion from clothing, topical steroid use. Differential diagnosis: pityrosporum folliculitis (b p.611). Management  Exclude DM; treat with topical antiseptic, or if not clearing with topical or systemic antibiotics (e.g. fusidic acid cream or oral flucloxacillin). If recurrent or chronic: treat as for recurrentboils.

Acute paronychia  Infection of the skin and soft tissue of the proximal and lateral nail fold, most commonly caused by Staph. aureus. Often originates from a break in the skin or cuticle as a result of minor trauma, e.g. nail biting. Skin and soft tissue of the proximal and lateral nail fold are red, hot, and tender; nail may appear discoloured/distorted. Treat in the same way as for aboil. Staphylococcal whitlow (felon)  Infection involving the bulbous dis-

tal pulp of the finger following trauma or extension from an acute paronychia. The finger bulb is red, hot, oedematous, and usually exquisitely tender. Onset of pain is rapid and there is swelling of the entire finger pulp. Differential diagnosis: herpetic whitlow—b p.635. Management  • If fluctuant Admit for drainage and antibiotics • If non-fluctuant Elevate, apply moist heat (e.g. soak in hot water), and treat with oral antibiotics; if this fails, admit for incision and drainage

Wound infection  Suspect if a wound becomes painful. Look for swelling, erythema, wound tenderness ± pus. Risk factors: • Malnutrition • Carcinomatosis • Infection near the site of incision • Steroid therapy • Contamination of thewound • DM Management  If pus is present send a swab forM,C&S: • If indurated + infection is localized to the wound suspect staphylococcus. Treat with flucloxacillin 500mg qds or clarithromycin 500mgbd • If cellulitis around the wound suspect streptococcus. Treat with penicillin V 500mg qds or clarithromycin 500mgbd • If foul smell, suspect anaerobes—treat with metronidazole 400mgtds

Give adequate analgesia; dress the wound frequently; review regularly; allow pus to drain. If a surgical wound, refer back to the operating surgeon if simple measures are ineffective. • Necrotizing fasciitis  Life-threatening soft tissue infection. Usually occurs in otherwise healthy individuals after surgery/trauma (often minor). Ill-defined erythema + high fever. The wound rapidly becomes necrotic. Admit as an emergency for IV antibiotics ± surgical debridement.

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Viral skin infection Systemic viral infections  bp.652 HIV infection  bp.746 Viral warts  Common and benign. Due to infection of epidermal cells

with human papilloma virus (HPV). >50 types identified. The virus is transmitted by direct contact. Immunosuppressed patients are particularly vulnerable. Genital warts  bp.748 Common warts  Dome-shaped papules with papilliferous surface. Usually >1. Most common on hands but may affect other areas. In children 30–50% disappear spontaneously in1. Manage as for common/ plantar warts. Eventually resolve spontaneously. May show Koebner phenomenon. Treatment of common, plantar, and plane warts  Refer immunosuppressed patients for specialist advice. Otherwise treatment is usually unnecessary. If patients are insistent, advise over-the-counter topical salicylic acid preparations, e.g. Duofilm®, Salactol®. HPV vaccination  bp.749

Herpes simplex infection  Herpes simplex virus (HSV) is transmitted

by direct contact with lesions. Lesions may appear anywhere on the skin or mucosa but are most frequent around the mouth and on the lips, conjunctiva, cornea, and genitalia. Diagnosis is usually clinical. Primary HSV stomatitis After a prodromal period (2 nails are involved. Confirm diagnosis with nail clipping mycology before treatment with oral terbinafine (250mg od for 6wk–3mo) or pulsed itraconazole (200mg bd for 7d, repeated after 21d, x2 for fingernail infections and x3 for toenail infections) • Scalp infection If kerion (pustular boggy mass) is suspected, refer to derma­ tology. Otherwise, oral terbinafine (250mg od) or griseofulvin (500mg–1g od) depending on sensitivities. • Griseofulvin may be teratogenic—advise 5 to avoid pregnancy during treatment and for 1mo afterwards and 4 to use contraception during treatment and for 6mo afterwards Pityriasis versicolor  bp.622 Table17.10  Dermatophyte infections Tinea

Affects

Presentation

Differential diagnosis

Corporis ‘Ringworm’

Trunk or limbs Single/multiple plaques with Discoid eczema scaling and erythema, especially Psoriasis at the edges. Lesions enlarge Pityriasis rosea slowly and clear centrally (hence ‘ringworm’)

Cruris ‘Jock itch’

Groin 4>5 Common in athletes

Associated with tinea pedis. Intertrigo Involves upper thigh (+ scrotum Candidiasis rarely). Red plaque with scaling Erythrasma especially at the edge

Pedis ‘Athlete’s foot’

Feet. 4>5 Young > old

Itchy, maceration between toes. Contact dermatitis Risk factors: swimming; occlusive Psoriasis footwear; hot weather Pompholyx

Capitis

Hair and scalp Defined, inflamed scaly areas ± alopecia with broken hair shafts

Unguium

Nails— prevalence i with age; rare in children. Toenails > fingernails

Alopecia areata Psoriasis Seborrhoeic eczema

Psoriasis Begins at distal nail edge and progresses proximally to involve Trauma Candidiasis the whole nail. Eventually results in thickening, yellowing, and crumbling of the nail plate. Tinea pedis often coexists

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Infestations Head lice Most common in children aged 4–11y (5 > 4)

but may occur in anyone. Contrary to popular belief lice infest clean as often as dirty hair. Adult lice are about the size of a sesame seed, brownish grey in colour and wiggle their legs (see Figure17.36). Only adults are contagious. Spread by close head–head contact. Lice do not jump/fly and do not remain viable away from ahost. Symptoms/signs  Normally asymptomatic. Detected by contact tracing of other cases or routine inspection at home or school. Occasionally present as itchy scalp. Presence of ‘nits’ (eggshells—white dots attached to hair), eggs, or dead lice indicate past infection—a moving louse must be found to confirm active infection. Detection  After washing hair, apply conditioner and comb with fine-tooth detector comb (available from pharmacy). In at-risk groups e.g. schoolchildren repeat weekly. Lice are removed by the comb and seen trapped in itsteeth. Management  Treat all household contacts simultaneously. • Prophylactic preparations No evidence of effectiveness • Dimeticone Lotion or spray. Coats lice and interferes with their water balance by preventing the excretion of water. Advise to rub into dry hair and scalp in the evening, allow to dry naturally, then shampoo off the next morning. Repeat after7d • Insecticides Effective. 4 types:malathion, phenothrin, permethrin (all available OTC, but NHS prescriptions are often sought), and carbaryl (prescription only). Malathion and phenothrin/permethrin are used as first-/second-line; carbaryl is reserved for third-line. Apply according to the manufacturer’s instructions using 2 applications 7d apart. Check wet, conditioned hair with a detector comb before the first application, then every 2d until 2–3d after the second application. Supply enough for 2 applications. Shampoos are ineffective—use lotions, liquids, or cream rinses • Mechanical clearance Wet-comb conditioned hair with a fine-tooth comb until all lice are removed and repeat at 3–4d intervals for 2wk. Alternative to insecticides but requires motivation • Other methods of treatment, e.g. electric combs, aromatherapy (tea tree oil), herbal treatments—no evidence supportinguse 0 If pregnant/breastfeeding, treat with wet-combing or dimeticone. Contact tracing  All cases—trace close contacts over the past month and ask them to check their scalps for lice/treat as needed. Reinfestation/resistance to treatment  3 possible reasons: • Reinfestation Lice found are large adults only. Ask patient to check close contacts again. Re-treat with a different insecticide • Incorrect use of insecticide/mechanical clearance Lice are at mixed stages of development. Check procedure and make sure instructions are understood. Repeat treatment with a different insecticide • Resistance to insecticide Lice are seen at all stages of development. Re-treat with another product

Infestations

Figure17.36  Head lice with needle and thread to give an idea ofsize

Crab (pubic) lice  bp.749 Scabies Extremely contagious. The scabies mite (Sarcoptes scabei) is

70.5mm long and spread by direct physical contact. Average infection consists of 12mites. Presentation  Symptoms of intense itching appear 4–6wk after infection. Examination reveals burrows (irregular, tortuous, and slightly scaly, 6wk for urgent further investigation.

Management in children Refer to paediatrics urgently, particularly if there is no evidence of local infection, if≥1of: • Non-tender, firm/hardLN • LN >2cm diameter • Progressively enlargingLNs • LNs associated with other signs of ill health (e.g. fever, weightloss) • Enlarged axillary nodes in the absence of local infection or dermatitis • Supraclavicular node involvement Investigate with FBC and blood film if generalized lymphadenopathy Table18.2  Normal temperature as measured in different locations Place of measurement

Normal range

Oral

35.5–37.5°C (95.9–99.5°F)

Rectal

36.6–38.0°C (97.9–100.4°F)

Axillary

34.7–37.3°C (94.5–99.1°F)

Ear

35.8–38.0°C (96.4–100.4°F)

Symptoms, signs, and notification of infectious disease

Pyrexia/fever Oral temperature raised above 37.5°C. Normal range varies according to where measured—see Table18.2. Common causes: Infection By far, the most common cause in general practice: • Sinusitis • Viral infection (e.g. HIV, • Chest infection • Cholecystitis EBV, URTI, influenza) • Tonsillitis • Cellulitis • OM • UTI

0 Do not forget tropical diseases, e.g. malaria in patients returning from abroad. Think of TB and SBE—especially in high-risk patients. Cancer Lymphoma; leukaemia; solid tumours (e.g. hypernephroma). Immunogeniccauses Connective tissue disease and autoimmune disease (e.g. RA, SLE, PAN, polymyalgia rheumatica); sarcoidosis. Thrombosis DVT;PE Drugs e.g. antibiotics

Fever in children under the ageof5 bp.874 Rigors Shaking episodes (sometimes violent) associated with sudden rise

infever.

Nightsweats Consider TB, lymphoma, leukaemia, solid tumour (e.g. renal carcinoma), menopause, anxiety states, drug causes, e.g. opioids,SSRIs.

Pyrexia of unknownorigin Defined as a fever (either intermittent

or continuous) which has lasted for >3wk and for which no cause has been found. Recheck history. Re-examine carefully. Check FBC; EBV screen (depending on age of the patient); ESR; CRP; LFTs; amylase, urine (M,C&S); viral titres (including HIV); blood cultures; and CXR. If cause does not become obvious refer urgently for further investigation.

Notifiable diseasesND Notification of certain diseases is required

under the Public Health (Control of Disease) Act 1984 and Health Protection (Notification) Regulations 2010. Notification is made to the ‘proper officer of the local authority’ on forms available from the HPA website (M www.hpa.org.uk). Diseases included: • Haemolytic uraemic • Mumps • Acute encephalitis • Plague syndrome • Acute infectious • Rabies • Infectious bloody hepatitis • Rubella diarrhoea • Acute meningitis • SARS • Acute poliomyelitis • Invasive group • Smallpox Astreptococcal • Anthrax • Tetanus disease and • Botulism • Tuberculosis scarletfever • Brucellosis • Typhus • Legionnaire’s • Cholera • Viral haemorrhagic disease • Diphtheria • fever • Leprosy • Enteric fever • Whoopingcough • Malaria (typhoid / • Yellowfever • Measles paratyphoid) • Meningococcal • Food poisoning septicaemia 0 In addition, notify other infections or contamination which could present significant risk to human health.

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Illness in returning travellers • In all returned travellers who present unwell, consider imported disease in addition to the usual differential diagnosis. Tropical medicine is a specialized field. If unsure, seek expert advice by telephone or admit the patient.

History Askabout: • Duration oftravel • Symptoms • Sexual contacts whilstabroad • Malaria prophylaxis • Immunizations prior totravel • Areas travelled to (including • Medical treatmentabroad brief stopovers) • Health of members of the travelparty Examination Full examination. Particularly check for fever, jaundice, abdominal tenderness, chest signs, rashes, lymphadenopathy. Investigations Depend on symptoms and examination findings. Consider: FBC, malaria testing (consult local protocols), LFTs, viral serology, blood culture, stool culture (ensure it is fresh),MSU. MalariaND 2,000 cases/y are notified in the UK. Easy tomiss. • Symptoms Malaria is a great mimic and can present with virtually any symptoms. Usually consists of a prodrome of headache, malaise, myalgia, and anorexia followed by recurring high fevers, rigors, and drenching sweats lasting 8–12h at atime • Examination May be normal—look for anaemia, jaundice ± hepatosplenomegaly • Investigation In all cases of fever in patients who have returned from a malarial endemic area—even if the plane just landed in a malarial area en route. Send a blood test for malaria daily for 3d. Tests include thick and thin film, dipstick or nucleic acid testing depending on local arrangements. • Management Admit for further investigation and treatmentif: • Malaria test+ve • Veryunwell • Persistent fever despite–ve • Unable to check a malaria malaria test test (e.g. presentation at a weekend or out ofhours) Falciparum malaria Caused by Plasmodium falciparum. Accounts for ~½ UK cases—it may not present for up to 3mo after return from a malarial area. Can be fatal in 5y

•  Give Hib/MenC and PPV immediately • 1mo after Hib/MenC and PPV, vaccinate with MenACWY

PCV7—pneumococcal conjugate 7-valent vaccine. PCV13—pneumococcal conjugate 13-valent vaccine. PPV—pneumococcal polysaccharide vaccine. Hib/MenC—Haemophilus influenza b/meningitis conjugate vaccine. MenACWY—meningitis quadrivalent (ACWY) conjugate vaccine.

0 Start vaccinations ≥2wk before splenectomy or starting immuno­ suppressive treatment.

Further information DH The Green Book:immunization against infectious disease. M www.dh.gov.uk/greenbook

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Childhood viral infections Table 18.4  Common childhood viral infections Condition

Duration Main symptoms

MeaslesND

10d

Incubation 10–14d Early symptoms Fever, conjunctivitis, cough, coryza, LNs Later symptoms Koplik’s spots (tiny white spots on bright red background found on buccal mucosa of cheeks), rash (florid maculopapular appears after 4d—becomes confluent) Complications Bronchopneumonia, otitis media, stomatitis, corneal ulcers, gastroenteritis, appendicitis, encephalitis (1:1,000 affected children), subacute sclerosing panencephalitis (rare)

RubellaND (German measles)

10d

Incubation 14–21d Symptoms Mild and may pass unrecognized. Fever, LNs (including suboccipital nodes), pink maculopapular rash which lasts 3d Complications Birth defects if infected in pregnancy; arthritis (adolescents); thrombocytopenia (rare); encephalitis (rare)

MumpsND

10d

Incubation 16–21d Symptoms Subclinical infection is common. Fever, malaise, tender enlargement of one or both parotids ± submandibular glands Complications Aseptic meningitis; epididymo-orchitis; pancreatitis

Chickenpox

14d

Incubation 10–21d (infectious 1–2d before rash appears and for 5d afterwards) Symptoms Rash ± fever. Spots appear in crops for 5–7d on skin and mucus membranes and progress from macule l papule l vesicle then dry and scab over Complications Eczema herpeticum (b p.606); encephalitis (cerebellar symptoms most common); pneumonia; birth defects; neonatal infection (b p.808)

Roseola infantum

4–7d

Child 5 x 109/L). Blood film—small lymphocytes, many of which are disrupted to form ‘smear’ cells. Management  Refer to haematology or discuss with a haematologist depending on age and clinical state of the patient. Once diagnosis has been confirmed, well patients with low levels of lymphocytosis are often managed in primary care with regular FBC and clinical review (at least every 6mo). Treat infections promptly. Refer for treatmentif: • Symptomatic disease (fevers, sweats, weightd) • Lymphadenopathy and/or hepatosplenomegaly • Rising lymphocyte count (i >50% in 2mo or doubling time of10y.

Chronic myeloid (granulocytic) leukaemia (CML)  Peak age:

30–60y. Chance finding in 20%. Otherwise presentswith: • Non-specific symptoms, e.g. weight d, lassitude, gout, anaemia • Splenomegaly (common)—abdominal pain, digestive symptoms, or pleuritic pain due to splenic infarction • Bleeding (rare)—due to abnormal platelet function Investigation  FBC: i WCC (usually >50 x 109/L) ± anaemia. Blood film: bone marrow precursors of myeloid cells (blasts). Cytogenetics: Philadelphia chromosome. Management Refer urgently to haematology. Treatment is determined by phase of the disease: • Chronic (90% at diagnosis)—30% of cells in the bone marrow are immature blasts—treated with chemotherapy

Myeloproliferative disorders  Proliferation of ≥1 of the haemo­ poietic components of the bone marrow. Includes: • CML • Essential thrombocythaemia • Polycythaemiavera • Myelofibrosis

Chronic leukaemia and myeloproliferation

Erythrocytosis  i in the number of circulating red cells. If haematocrit

is persistently i for >2mo (>0.524; >0.485), investigate the cause. May be s (polycythaemia vera) or s. 0 Hb may also appear i if dehydrated (concentration effect). Secondary polycythaemia maybe: • Appropriate High altitude, chronic lung disease (e.g. COPD), cardiovascular disease with a right l left shunt, heavy smoking, sleep apnoea, i affinity for haemoglobin (familial polycythaemia),or • Inappropriate Caused by excess erythropoietin, e.g. s to hepatocellular or renal tumour, or massive uterine fibroid. May need venesection

Polycythaemia vera  Also known as primary proliferative polycythaemia (PPP). Haematological malignancy resulting in overproduction of red cells. Age range: most>50y. Presentation  Non-specific symptoms/signs: • Headaches, dizziness, vertigo, • Nightsweats • Dusky, cyanotic hue with red face and/or tinnitus • Itching (especially provoked by • Gout—s to i red cell turnover water, e.g. after abath) • Peptic ulceration (5–10%) • Splenomegaly (70%) ± hepatomegaly • Thrombosis/haemorrhage—abnormal platelet function/hyperviscosity Investigation  Often diagnosed incidentally following FBC done for other reasons—persistently i Hb + haematocrit; neutrophils and platelets may also be i. JAK2 mutation is +ve in>95%. Management  Refer urgently to haematology. Hb level is d by regular venesection ± cytotoxics. Aspirin d risk of thrombosis. Slowly progressive and survival for 10–20y is not unusual; a minority eventually transform to acute myeloid leukaemia or myelofibrosis.

Essential thrombocythaemia  Patients have i risk of thrombosis, but

may haemorrhage due to abnormal platelet function. FBC—persistently i platelet count >450 x 109/L when reactive and other myeloproliferative causes have been excluded. Refer urgently to haematology. Treatment is with aspirin, treatment of CVD risk factors ± cytotoxics. May eventually transform toAML.

Myelofibrosis (myelosclerosis)  Progressive accumulation of fibrous

tissue in the bone marrow cavity, replacing normal marrow. Haemopoietic function is taken over by the spleen/liver. Patients are usually elderly and present with symptoms of anaemia, malaise, fever ± gout. The spleen is massively enlarged FBC—d Hb; blood film—immature erythroid cells (normoblasts) and myeloid cells (metamyelocytes/myelocytes). Red cells are teardrop-shaped. Refer urgently to haematology. Median survival is 2–3y— but many live much longer. 5–10% transform toAML.

Further information

British Committee for Standards in Haematology M www.bcshguidelines.com • Investigation and management of adults and children presenting with thrombocytosis(2010) • Diagnosis, investigation, and management of polycythaemia/ erythrocytosis (2005)

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Lymphoma Cancer of the lymphatic system. Two maintypes. Non-Hodgkin’s lymphoma (NHL)  Derived from malignant transformation of lymphocytes—85% B cells. Usually develops in LNs but can arise in any tissue. Incidence: 12,300 cases/y in the UK (4% cancers), causing 4,500 deaths/y. 4 = 5. 69% occur in patients>60y. Presentation  May be detected incidentally on CXR (mediastinal mass) or present with painless peripheral lymphadenopathy; abdominal mass (nodal or spleen); weight d; night sweats/unexplained fevers. Other symptoms are dependent on site, e.g. neurological symptoms if CNS involvement; pleural effusion; skin lesions. Investigation  FBC—may be normal if no bone marrow involvement; monospot—perform in all patients 2cm insize • Widespread lymphadenopathy • Lymphadenopathy + weight d, night sweats, and/or splenomegaly • Any other suspicious symptoms/signs Specialist treatment Based on histology and stage. Treatment options include a wait-and-see approach for low-grade lymphomas; radiotherapy; chemotherapy; bone marrow transplant; monoclonal antibody therapy (rituximab); and/or immunotherapy. Prognosis  Varies widely between different types of NHL (Table 19.7) and the age of the patient. Younger, fitter patients with less widespread disease do better. Overall 51% survive10y.

Hodgkin’s lymphoma  1,900 cases/y in the UK. Peak age ranges: 15–35y (>50% occur 5. Usually presents at a late stage and with systemic symptoms Cutaneous form5% NHL. Affects adults (peak age 61y). Presents with reddish brown skin nodules or ulceration ± regional LN involvement (25%) Affects children—30–40% childhood lymphoma. 4 > 5. B-cell lymphoma. Two varieties. Endemic variety is more common in Africa and associated with EBV infection. Peak age 5–10y. Sporadic variety occurs worldwide and affects slightly older children Presents with bulky central nodal disease ± extranodal (typically abdomen), bone marrow, and/or CNS involvement

Low-grade NHL Follicular

Small lymphocytic

Mantle cell

Marginal zone

Lymphoplasmacytic

Affects adults. B-cell origin. Three types divided according to the ratio of small and large cells Usually presents with disseminated disease. 50% present with bone marrow involvement. May transform to DLBCL 4–5% NHL. Median age 60y. Clinically/morphologically identical to CLL (b p.678). Distinguished by degree of lymph tissue vs. blood/bone marrow involvement Presents with diffuse lymphadenopathy and some blood/bone marrow involvement. 10–20% transform to CLL, 3% to DLBCL 5% NHL. Affects adults usually >50y. 4 > 5 (4:1). Although classified as low grade, behaves and is treated as high-grade. Usually presents with widespread disease involving LNs, bone marrow (60–90%), peripheral blood, spleen ± gut. Poor prognosis B-cell origin. Three distinct types Nodal 1–3% NHL. Presents with localized lymphadenopathy Splenic 4wk) or shedding of primaryteeth • Failure tothrive • Adverse reaction to immunization or viral infection • Difficult-to-treat asthma oreczema • Splenectomy, tonsillectomy, or adenoidectomy • Prior prophylactic antibiotic or immunoglobulin therapy Primary immunodeficiency As many p immunodeficiencies are hereditary or congenital, they appear initially in infants and children; 780% of those affected are >5. Genetic screening is available for some conditions. Refer all suspected cases to paediatrics/immunology. Classification  >70 primary immunodeficiencies have been described. They are classified into 4 groups depending on which component of the immune system is deficient: • Bcells • Phagocyticcells • Tcells • Complement Prevalence  Selective IgA deficiency (usually asymptomatic) occurs in 1:400 people. All other primary immune deficiencies are rare. Excluding IgA deficiency, 50% of affected patients have B-cell deficiency; 30% T-cell deficiency; 18% phagocytic deficiencies; and 2% complement deficiency. Presentation  Table19.8 lists some of the more common immune deficiencies. All immune deficiencies present with increased tendency to infections. Type of infection varies according to the component of the immune system involved. If suspected, refer for specialist review.

Secondary immunodeficiency  Impairment of the immune system resulting from illness (including drug therapy, e.g. with cytotoxics or steroids) or removal of the spleen in a previously normal person. Often reversible if the underlying condition or illness resolves. s immunodeficiencies are common; most prolonged serious illness interferes with the immune system to some degree. Treat thecause. HIV infection  bp.746 Asplenia and splenectomy  bp.650 Infection in the immunocompromised  bp.650 Information and support for patients Immune Deficiency Foundation M www.primaryimmune.org

Immune deficiency syndromes

Table19.8  Immune deficiency syndromes Type

Syndrome

B-cell deficiency Prone to infection with Gram +ve organisms (e.g. streptococci)

Selective IgA deficiency and IgG subclass deficiencies

T-cell deficiency Prone to viral, fungal, and opportunistic infections

Combined B- and T-cell deficiency

Phagocytic deficiency Prone to staphylococcal and Gram–ve infections

Clinical details

Variable symptoms with most only mildly affected When more severely affected, early treatment of infection may be required Not inherited—cause unknown Congenital X-linked hypogammaglobulinaemia and d immunoglobulins Common variable Treatment is with IV immunoglobulin immunodeficiency i risk of leukaemia /lymphoma di George’s syndrome Defect on chromosome 22 l absent/ hypoplastic thymus (and d Tcells), absent parathyroid glands ± cardiac and/or facial abnormalities – Mild (80%)—treated supportively – Severe—requires thymus/bone marrow transplant HIV b p.746 Severe combined Autosomal or X-linked recessive immunodeficiency Absence of both T-cell and B-cell immunity Presents 50y Two-view mammographic screening is currently available to women aged 50–70y every 3y. This will be extended to women aged 47–73y throughout the UK by 2016. Older women can also request screening every 3y. Screening detects 85% of cancers in women aged >50y (60% of which are impalpable) and ~70–80% screening-detected cancers have good prognosis. Screening more frequently does not d mortalityR. Organization of breast cancer screening in the UK—see Figure20.2. High-risk women8%; women aged 40–49y with 10y risk >20%; and at-risk women aged 40–49y with a dense breast pattern on mammography

Interval cancers Cancer occurring in the interval between screens. Can occur through failure to detect a cancer at screening or as a result of a new event after screening took place. In the first year after screening 20% breast cancers are interval cancers. This i to 760% in the third year. Acceptability of screening 81% women find mammography uncomfortable, but 90% return for subsequent screens. GPs have an important role in promoting National Cancer Screening Programmes to their patients; GP endorsem*nt i uptake ratesR.

Anxiety from screening False-positive results cause anxiety as well as prompting further invasive investigations. Anxiety levels in women recalled and then found to be disease-free are higher a year after the recall appointment than in women who receive negative results at screening. Further information NHS Breast Screening M www.cancerscreening.nhs.uk NICE Familial breast cancer (2006) M www.nice.org.uk Information for patients M www.cancerscreening.nhs.uk • Breast screening—thefacts • Over 70? You are still entitled to breast screening

Breast cancer screening

All women aged 50–70y (47–73y by 2016) Invited to screening Screening with two-view mammography at a screening centre

Normal screen

~75% of women invited

Abnormal screen ~5% of women screened*

3-year recall Immediate recall for further investigation

All clear Early recall in 6mo or 1y * 8% first-time screens; 4% subsequent screens

Diagnosis of breast cancer 0.6% of women screened 10% of women recalled

Figure20.2  Organization of breast cancer screening intheUK

Table20.2  Pros and cons of breast cancer screening Benefits

Adverse effects

•  Earlier diagnosis • Improved prognosis

•  Discomfort and inconvenience of screening •  Radiation risks of screening (verysmall) • Reassurance to those women who have

andlower mortality

• Less radical and

invasivetreatment needed • Reassurance for thosewith –ve results

false–ve results

• Reassurance to those who develop an interval

cancer and possibly later presentation due to false sense of security • Anxiety and adverse effects of further investigation for those with false+ves • Overdiagnosis of minor abnormalities that would never develop into breastcancer • Earlier knowledge of disease and overtreatment for those who, despite early diagnosis have unchanged prognosis

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Breastcancer Breast cancer is now the most common cancer in the UK with >48,000 new diagnoses every year (including 340 new cases/y affecting men). Women have a 1 in 8 lifetime risk of developing breast cancer. Virtually all breast cancers are adenocarcinoma (85% ductal; 15% lobular).

Risk factors Geography More common in the developed world—migrants assume the risk of the host country within two generations. Personal characteristics • Age i with age—781% of breast cancers occur in women>50y • Socio-economic Higher incidence in more affluent social classes • Physical characteristics Taller women have i risk; women with denser breasts have 2–6x irisk Lifestyle factors • Obesity i risk post menopause • Physical activity 30% d risk if taking regular physical activity • High-fat diet Probably associated withirisk • Alcoholi risk by 7%/unit consumed/d Reproductive history • Early menarche or late menopause irisk • Pregnancy i parity results in d risk (32% d risk in women reporting three births compared to women reporting one); late age when first child is born irisk • Breastfeeding d relative risk by 4.3% for each year of breastfeeding • Combined hormonal contraception Slight i risk (relative risk 1.24 for current users)—excess risk disappears within 10y of stopping • HRT Risk i by 6 cases/1,000 after 5y combined HRT use and 19 cases/1,000 after 10y use. Risk for combined oestrogen and progestogen preparations is greater than oestrogen-only preparations. HRT also d sensitivity of mammography Other past medical history • Past history of breast disease Ductal or lobular carcinoma in situ, florid hyperplasia, and papilloma with fibrovascular core all irisk • Ionizing radiation Exposureirisk Family history Referral algorithm—see Figure20.3. • One first-degree relative with breast cancer (mother/sister) Risk i 2x—but 95% of women with breast cancer have no family history • Several family members with early onset breast cancer Refer for genetic screening—BRCA1 and BRCA2 genes account for 2–5% of all breast cancers 0 Family relationships: • First-degree relative—mother, father, sister, brother, daughter,son • Second-degree relative—grandparents, grandchildren, aunt, uncle, niece, nephew, half-sister, half-brother

Breast cancer screening bp.694

Breastcancer

Concerns about family history of breast cancer Refer for genetic counselling

YES

Has a faulty gene been identified in the family?

Manage in 1° care

NO Is there a history of breast cancer in any 1st or 2nd degree maternal or paternal relatives? YES Is there ONLY ONE relative diagnosed with breast cancer >40y? NO Does the patient have ≥1 high-risk criterion?

NO

NO

Are there any unusual cancers in the family (bilateral/male breast cancer; ovarian cancer; sarcoma 2mo • If mild/moderate symptoms—try lifestyle/dietary modificationfirst: • Make allowances on days when symptoms are likely to beworst • Wear loose clothes if feeling bloated • Ensure adequate sleep and take regular exercise • Eat regularly—some find small, frequent meals help; avoid sweet snacks between meals; make sure diet is low in fat/salt, caffeine, and alcohol, and contains plenty of fruit/vegetables and complex carbohydrate (e.g. bread, pasta, rice, potatoes) • d fluid intake or eat diuretic foods (e.g. strawberries, watermelon, aubergines, prunes, figs, parsley) to ease fluid retention • OTC remedies may help (see Table21.1) • Consider drug therapy or CBT (see Table21.1) if symptoms are severe or do not respond to diet/lifestyle measures. Base choice on symptoms • For all treatments try a 3–6mo trial. Ask women to keep a symptom diary. Be sure to follow up—as the first treatment may notwork • If symptoms are severe or primary care management is ineffective, refer to gynaecology/mental health services for specialist management 0 Because of side effects, oestrogen patches and gonadotrophin-releasing hormone analogues are usually only used by specialists for severe or resistant PMS/PMDD.

Further information RCOG Management of premenstrual syndrome (2007) M www.rcog.org.uk

Information and support for patients and their partners National Association for Premenstrual Syndrome F 0844 8157311 M www.pms.org.uk

Premenstrual syndrome

Table21.1  Treatment of premenstrual tension Treatment

Effective? Notes

Hormonal manipulation COCG

First-line treatment. The RCOG recommends combined new generation pills, e.g. Yasmin® or Cilest®—given cyclically or continuously

Low-dose oestrogenG

Second-line treatment. Use 100 microgram estradiol patches. To avoid giving unopposed oestrogen, combine with dydrogesterone 10mg from day 17–28 or IUS if the uterus is intact

GnRH analoguesR

Reserved for specialist care. Usually given in combination with add-on HRT

d physical as well as psychological symptoms. First-line treatment that can be given continuously or just in the luteal phase (days 15–28)

DiureticsCE

Spironolactone is effective for bloating/breast tenderness. Many women prefer ‘natural’ diuretics, e.g. Waterfall®, though there is no evidence of effectiveness

NSAIDsCE

Particularly helpful for premenstrual pain and to d menstrual bleeding

Curative. Most women require HRT/ testosterone replacement afterwards

Antidepressants SSRIsG

Other drugs

Surgery Hysterectomy + oophorectomyG

Complementary therapies Oil of evening primroseS

/

May help breast tenderness. Can cause fits in patients with epilepsy

Vitamin B6G

Most studies suggest effective—advise women to take 10mg/d. High doses (>100mg/d) may cause reversible peripheral neuropathy

Chaste tree berry (Vitex agnus-castus) S

Evidence of effectiveness is generally positive— can cause menstrual irregularity

Magnesium supplementsG

Evidence of effectiveness is generally positive. Used in the premenstrual phase

Calcium supplementsS

d symptoms including breast tenderness and swelling, headaches, migraine, and abdominal cramps

ExerciseCE

High-intensity exercise improves symptoms >low-intensity exercise

Cognitive therapyCE

Evidence that effective. Effects are smaller and slower than SSRI—but more long-lasting

Relaxation/ reflexologyR

/

Conflicting evidence—can do no harm

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Amenorrhoea Oligomenorrhoea Infrequent periods (>35d between periods). Manage as for amenorrhoea. Primary amenorrhoea No menstruation by age 16y when growth and sexual development is normal. Refer for specialist treatment. Causes: • Outflow abnormalities Müllerian agenesis; transverse vagin*l septum; androgen insensitivity (b p.895), imperforatehymen • Ovarian disorders PCOS; gonadal dysgenesis, e.g. Turner’s syndrome—0 gonads may have malignant potential • Pitutary disorders Prolactinoma (b p.370) • Hypothalamic disorders Kallman’s syndrome (congenital GnRH deficiency associated with anosmia) Secondary amenorrhoea Absence of menses for ≥3mo in a previously menstruating woman. Causes: see Figure21.2.

History  Always consider the possibility of pregnancy. • Symptoms • Galactorrhoea—30% prolactinomas • Weight change—weight d may cause amenorrhoea • Hirsutism—may suggest PCOS or androgen-secreting tumour • Life crisis or upset—e.g. exams, bereavement • Level of exercise—high-intensity athletes, e.g. gymnasts are frequently amenorrhoeic • Sweats and/or flushes (suggests menopause) • Cyclical pain—may suggest outflow obstruction • Family history Of premature menopause or late menarche • Drug history Particularly contraceptives, e.g. injectable progestogens. Other drugs include:heroin, methadone, metoclopramide • Past history Of chemo- or radiotherapy or gynaecological surgery Examination • Weight and height—common if BMI 45y with new menorrhagia or not responding to treatment)

Figure21.3  Assessment of menorrhagia in primarycare

Menorrhagia

Management  See Figure21.4. Treatment may fail if: high blood loss; low pre-treatment loss; other uterine pathology; lack of concordance. 2º care management: assessment of endometrium; IUS; surgery—endometrial resection and ablation, myomectomy, hysterectomy ± oophorectomy. Heavy menstrual bleeding and no sinister pathology suspected—consider IUS (b p. 762). Check: no contraindications and ≥12mo use is anticipated Blood flow still too great

Blood flow not d to acceptable levels after >6mo use

Add CHC if no contraindications

Blood flow reduced to acceptable levels

Contraindications to IUS, 6mo

Tranexamic acid 1g tds

Requires contraception

Try CHC (b p. 752). Review after 3mo

Continued Add heavy bleeding

Start on day 1 of period, and continue for days of heavy flow. Review after 3mo

Continued heavy bleeding. Try norethisterone 15mg od from day 5–26 of the menstrual cycle, or injected long-acting progestogen

Blood flow not reduced to acceptable levels after 3mo—refer

1 Management of very heavy bleeding • Resuscitate as necessary—admit if shocked; IV tranexamic acid or D&C in the acute situation can d haemorrhage by 75–80%. • Reduce/stop bleeding with progestogen, e.g. norethisterone 5mg tds, for 10d. Effective in 24–48h. A lighter bleed follows on stopping. Alternatively consider tranexamic acid (1g tds for 4d) to d bleeding. • Correct anaemia and refer for gynaecology assessment.

Figure21.4  Medical management of menorrhagia in primarycare

Further information NICE Heavy menstrual bleeding (2007) M www.nice.org.uk

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The menopause From the Greek meno (month) and pausis (halt). Menopause occurs when menstruation stops. Average age in the UK 751y. Smoking brings it forwards by 72y. Impact on a woman’s life varies and depends on cultural, health, and social factors.

Diagnosis  >12mo amenorrhoea with no othercause. Period changes • Changes in menstrual pattern Common in the years before the menopause—typically cycle shortens after 40y by up to 7–10d. Cycle then lengthens—periods may occur at 2–3mo intervals until stopping • Dysfunctional uterine bleeding Common leading up to the menopause but investigate if very heavy, painful, irregular, intermenstrual, or post-coital bleeding • Late menstruation (>54y) Investigate as i risk of malignancy

Psychological symptoms  Controversial. Some studies report depression/anxiety are more common, others find no association. Depression is multifactorial—consider social, physical, and cultural factors before resorting to HRT as a solution. Regular sustained aerobic exercise (e.g. swimming or running) d psychological symptoms and insomnia. Flushes and sweats 80% have flushes during the menopause—20% seek help. Often associated with palpitations. Lifestyle changes  Low-intensity exercise (e.g. yoga, deep breathing); cool ambient temperature; wearing natural fibres, e.g. cotton; stress d; avoiding trigger foods/drinks (e.g. spicy foods, caffeine, alcohol). Drug treatments  • Hormone therapy (b p.712)—effective in 80–90% ofwomen • Megestrol acetate (40mg od—unlicensed)—effective but carries many of the same risks as HRT—may cause vagin*l bleeding on withdrawal • SSRIs/SNRIs (e.g. venlafaxine 37.5mg bd—unlicensed)—moderately effective and may also improve mood • Gabapentin (300mg tds—unlicensed)—d flushes by45% • Clonidine—commonly used but little evidence of effectiveness; may cause hypotension Complementary therapies  • Black cohosh eases hot flushes—long-term effects are unknown • Red clover may help—studies have mixed results; avoid with warfarin • Foods containing phyto-oestrogens (e.g. soy foods) may be helpful and are unlikely to be harmful • Natural progesterone from yams—topical preparations are ineffective • Dong quai, evening primrose oil, vitamin E, and ginseng are no better than placebo. Avoid kava—it is linked to cases of serious liver damage

Sexual dysfunction  vagin*l dryness and atrophy are common. Manage with vagin*l lubricants or topical oestrogen (unless other reasons for systemic HRT). Loss of libido (especially after surgical removal of the ovaries) responds to administration of androgen (e.g. testosterone) with HRT, until libido is re-established.

The menopause

Urinary problems Common—incontinence, nocturia, and urgency. Stress incontinence does not respond to HRT but topical oestrogen may improve outcome of surgery. Recurrent UTIs and incontinence in older women d with use of topical vagin*l oestrogen.

Ischaemic heart disease Risk is i x2 after the menopause but there is no evidence to support use of HRT for p or s prevention ofIHD.

Osteoporosis Consider HRT to prevent osteoporosis in premature

menopause (b p.511). In older women, HRT is not recommended as first-line treatment of osteoporosis unless there are other reasons for prescribingHRT.

Could the symptoms be due to another cause? Exclude: • Physical illness, e.g. thyroid disease, anaemia, DM, chronic renal disease • Side effects of medication, e.g. Ca2+ antagonists cause flushing • Social problems or psychiatric illness—depression screening questionnaires can be helpful Is the diagnosis in doubt?  FSH >30iu/L on 2 occasions >1mo apart

suggests the woman is postmenopausal. CheckFSH: • Following hysterectomy with conservation of ovaries • If amenorrhoea age 45y with amenorrhoea. FSH levels may be normal in the perimenopause.

Premature menopause  Menopause in a woman 5y No d risk of CHD—may i risk in the first year of use

Alleviation of menopausal symptoms, e.g. flushes/ sweats/ vagin*l dryness d Recurrent UTIs

d Osteoporosis d Colorectal cancer

 These figures are controversial. Subsequent studies, including long-term studies, have shown beneficial effects of HRT on symptoms, mortality, and CVD risk with no significant adverse effectsR

ψ

Relative risk in women aged 50–64y using combined HRT for5y Relative risk in women aged 60–69y taking combined HRT for5y

*

Further information British Menopause Society M www.thebms.org.uk RCP (Edinburgh) Consensus conference on HRT:Final consensus statement (2003) M www.rcpe.ac.uk/policy-standards/rcpe-consensusstatements Schierbeck LL, Rejnmark L, Tofteng CL, et al. (2012) Effect of HRT on cardiovascular events in recently post menopausal women:randomized trial. BMJ 345:e6409.

Information and support forwomen Menopause Matters M www.menopausematters.co.uk

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Pelvicpain Pelvic pain may be acute or chronic (pain for ≥6mo). Causes: see Table21.3.

History  Allow the woman to tell her story. Askabout: • Pain—site; severity; onset (? pregnant); character/timing/pattern (e.g. relationship to menstrual cycle or sexual intercourse, exacerbating/ relieving factors, effects of movement/posture); other associated features. Decide whether the cause is gynaecological—patients usually have dyspareunia and pain may be cyclical • Bowel (nausea and vomiting)/bladder/psychological symptoms • Past history—ectopic pregnancy, pelvic infection/surgery, other factors (be sensitive to possible history of sexual abuse orrape) Examination Abdominal, pelvic, and vagin*l examination—including rectal examination if indicated and cervical smear if overdue. Normal pelvic and vagin*l examination makes a gynaecological cause unlikely. Investigation Consider:urine—pregnancy test, M,C&S, dipstick for protein, RBCs, nitrites, and leucocyte esterase; blood—FBC, CRP, CA125; radiology—pelvic USS if gynaecological cause is suspected. 0 Offer screening for STIs to all sexually active women with chronic pelvic pain. Management of acute pelvic pain Admit if severe or if ectopic pregnancy cannot be excluded. Otherwise provide analgesia and treat the cause.

Management of chronic pelvic pain  Affects 1 in 6 women in the UK. There is often >1 cause—aim to identify and address contributory factors instead of assigning a singlecause. Dyspareunia  Pain on intercourse. 10% women admit sexual intercourse usually causes discomfort. It may be superficial (felt around the introitus) or deep (felt deep inside). There is a psychological element in most cases (a vicious cycle of pain leading to fear of intercourse which exacerbates symptoms). Address both physical and psychological aspects. Superficial dyspareunia  Examine if possible but do not insist. Treat cause. If no specific treatment, try lidocaine gel. Causes: • Vulval Vulvitis—atrophic, infective (candida, HSV); dystrophy; neoplasm; lichen sclerosus; lichen planus; vulvodynia • vagin*l Vaginismus; lack of lubrication; vaginitis—atrophic, infective; congenital—imperforate hymen, atresia; surgery, e.g. painful episiotomy scar; contracture—atrophy or after surgery/radiotherapy • Urethral Urethritis; urethral caruncle; urethral diverticulum Deep dyspareunia Causes: endometriosis; pelvic inflammatory disease; retroverted uterus; ovarian mass (rarely ovarian cancer); non-gynaecological causes. Examine, treat any cause found else refer for further investigation. If no cause is found or cause is untreatable, pain can be d by limiting penetration. Often becomes a chronic problem. Dysmenorrhoea (painful periods) >50% premenopausal women have some pelvic discomfort around their period; 1 in 10 find period pain significantly interferes with lifestyle.

Pelvicpain

Primary dysmenorrhoea  No underlying pelvic pathology. Tends to start 6–12mo after menarche when ovulatory cycles are established. Presents with lower abdominal cramps ± backache which occur in the first 1–2d of each period. May be associated GI disturbance, e.g. diarrhoea/ vomiting. Young women (3y; depo-medroxyprogesterone acetate injection is an alternative • If a woman is not trying to conceive and there is no evidence of a pelvic mass, try a progestogen (e.g. norethisterone 10–15mg/d for ≥4–6mo—starting on day 5 of each cycle—if spotting occurs i dose to 20–25mg/d and stop once bleeding has ceased) or continuous CHC (3 or 4 packets without a break then 7d break) • If symptoms are not controlled—refer

Endometriosis and adenomyosis

Specialist treatments • Medical options Include gestrinone and GnRH agonists (e.g. goserelin) ± HRT (d side effects and bone demineralization). Side effects can be troublesome • Surgical options Include laparoscopy or laparotomy with ablation of lesions and division of dense adhesions; tubal surgery; hysterectomy. Laparoscopic ablation of mild endometriosis may i fertility 0 For all forms of specialist treatment there is a 15–20% recurrence rate. If relapse in 6mo, consider the condition to have relapsed and repeat treatment. Psychological support  Many women will have had pain for years. Often there is delay in diagnosis of the cause—and, frequently, they have been told it is psychosomatic. Be sympathetic and supportive and use a cooperative strategy for management.

Adenomyosis Usually affects multiparous premenopausal women aged >35y. Caused by extension of endometrial tissue and stroma into the uterine myometrium. May coexist with endometriosis (15%) but a separate entity. Presentation  May be asymptomatic, or present with dysmenorrhoea (pain often peaks towards the end of menstruation), dyspareunia, and menorrhagia. On examination, the uterus may be symmetrically enlarged and tender. Management  No treatment needed if asymptomatic. Refer for further investigation of symptoms. MRI may confirm diagnosis but diagnosis is often only confirmed on histology after hysterectomy. Treatment is usually surgical with hysterectomy ± bilateral salpingo-oophorectomy. Medical treatment with GnRH analogues is a short-term option but symptoms return once withdrawn unless the woman has reached the menopause in the interim.

 Pelvic venous congestion Chronic pelvic pain due to dilation

and congestion of pelvic veins. Remains a controversial diagnosis. There is no diagnostic test; refer to exclude PID and endometriosis. Treatment is with progestogens, e.g. medroxyprogesterone 30mg od for 6mo, or GnRH analogues, e.g. goserelin 3.6mg monthly for 6mo. Symptoms often recur following treatment.

Further information RCOG M www.rcog.org.uk • The investigation and management of endometriosis (2006) • The initial management of chronic pelvic pain (2012)

Information and support for patients Endometriosis UK F 0808 808 2227 M www.endo.org.uk Pelvic Pain Support Network M www.pelvicpain.org.uk

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Prolapse Prolapse  Pelvic organs herniate into the vagin* due to poor pelvic muscle tone and weakness of pelvic ligaments. Affects 12–30% of multiparous and 2% of nulliparous women. Good obstetric practice d risk. Risk isiby: • Childbirth • Coughing and straining • Menopause • Congenital connective tissue disorders Terminology  Named according to the organs involved: • Cystocoele—bladder bulges into the vagin* • Urethrocoele—urethra bulges into the vagin* • Rectocoele—rectum bulges into the vagin* • Enterocoele—loops of intestine bulge into the vagin* • Uterine—uterus descends into the vagin* Uterine prolapse is further classified by degree. The most dependent portion of the prolapse is assessed whilst straining: • 1st degree prolapse–the cervix remains in the vagin* • 2nd degree prolapse–the cervix protrudes from vagin* on coughing/ straining • 3rd degree prolapse (procidentia)–the uterus lies outside the vagin* and may ulcerate Presentation  Dragging sensation, feeling of ‘something coming down’, or a ‘lump’. Symptoms are worse when upright, i.e. whilst awake, and exacerbated by standing for a long time, coughing, or straining. Associated symptoms  Depending on structures involved—stress incontinence (b p.450), difficulty defecating, recurrent cystitis, and/or frequency of micturition. In severe cases renal failure may occur due to ureteric kinking. Examination/investigation  Check abdominal examination to exclude pelvic masses. Then in left lateral position with Sims speculum, ask the patient to bear down and watch the vagin*l walls. Exclude pelvic mass by bimanual examination. Dipstick urine ± send forM,C&S. Management  Choice of treatment depends on patient preference, general health, degree of prolapse, severity of symptoms, and wish to preserve fertility and sexual activity. Options include: • Lifestyle measures Weight d; smoking cessation • General measures Treatment of coexisting conditions exacerbating prolapsed, e.g. chronic cough due to COPD or asthma, constipation, menopause/atrophic vaginitis • Physiotherapy Pelvic floor exercises (b p.841). Refer to specialist physiotherapy if simple self-help techniquesfail • Ring pessary Useful for those too frail for surgery, women who have symptoms but do not want surgery or as a temporary measure whilst awaiting surgery. Change pessary every 3–6mo. Shelf pessaries may be useful for women who cannot retain a ring pessary—consider referral • Surgery Refer to gynaecology if the woman is fit for surgery and symptoms are of sufficient severity to warrant operation and/or she has incontinence and/or recurrent UTI. Surgical options include repair operations (anterior or posterior colporrhaphy), colpo-vagin*l suspension, and hysterectomy (vagin*l or abdominal)

Prolapse

Fitting a ring pessary • Measure the approximate size required manually—the distance between posterior fornix and pubic bone can be measured roughly against the index finger • Soften the ring in hot water and lubricate itwell • Insert the ring into the posterior fornix and tuck it above pubicbone • Change the pessary every 3–6mo. Inspect the vagin* for damage (e.g. ulceration) before inserting the newring Potential problems • Discomfort Ring may be too big (try smaller size) or atrophic vaginitis (try topical oestrogen) • Infection Remove, clear infection then tryagain • Ulceration Remove, allow to heal, consider alternatives or reinsert when fully healed • Expulsion Ring may be too small, pelvic musculature indequate, or retropubic rim unsuitable

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Uterine problems Postmenopausal bleeding (PMB)  Perform a pelvic examination, including speculum examination of the cervix. Refer urgently (to be seen in 4mm on USS done for anotherreason Differential diagnosis  Atrophic change (most common); endometrial hyperplasia; endometrial polyps; endometritis; endometrial malignancy (10% referred); cervical malignancy; fibroids; uterine sarcoma; non-genital causes (bladder and bowel). Pelvic or abdominal mass Refer urgently for USS if abdominal/pelvic mass that is not clearly uterine fibroids and not of GI/urological origin. If USS is suggestive of cancer or urgent USS is not available, refer urgently. Congenital abnormalities of the female genitaltract • Duplication Of the cervix and/or uterus; vagin*l septum; bicornuate uterus (of varying degrees). Usually found incidentally. May cause problems in pregnancy/with IUD. Refer if needed • Imperforate hymen May cause cryptomenorrhoea which presents as p amenorrhoea (b p.706). Refer for surgical release if suspected • Ambiguous genitalia b p.895 • Cervical incompetence b p.816

Uterine retroversion 20% have a retroverted, retroflexed uterus. May be difficult to palpate bimanually—push on the cervix to antevert. Fibroids (uterine leiomyoma) Benign tumours of the smooth

muscle of the myometrium affecting 1 in 5 women. Often multiple. Oestrogen-dependent so regress post-menopause. Named by location: • Subserosal (bulge into peritoneum) • Pedunculated • Submucosal (bulge into endometrium) • Intramural (centrally in • Separate from uterus, especially in broad myometrium) ligament from embryonal remnants • Cervical Risk factors  Nulliparity; obesity; FH of fibroids; African origin. Presentation  Usually asymptomatic. Maycause: • Pelvic pressure/discomfort and/or backache • Menorrhagia—usually submucous fibroids distorting endometrial cavity • Pain: torsion (pedunculated fibroid); degeneration. Red degeneration may occur in pregnancy (pain, fever, and local tenderness) • Urinary symptoms—may press on the bladder li frequency or a feeling of incomplete emptying or difficulty passingurine • Infertility—may act as a ‘naturalIUCD’ • Problems in pregnancy—abnormal lie and i risk of post-partum haemorrhage. Risk of miscarriage is noti • Bulky uterus ± pelvic mass felt abdominally Pelvic USS is diagnostic. Check FBC if menorrhagia. 0 Calcified fibroids may be an incidental finding onX-ray.

Uterine problems

Management  If asymptomatic/mild symptoms—no treatment needed. Otherwise, management depends on presence of symptoms, number and location of fibroids, fertility plans, and patient’s preferences: • Medical—CHC or IUS d menstrual loss; GnRH analogues (maximum use 6mo due to risk of osteopenia) and selective progesterone receptor modulators (e.g. asoprisnil) cause fibroid shrinkage • Surgical—Uterine artery embolization; myomectomy (removal of fibroids only); hysteroscopic resection; hysterectomy

Endometrial proliferation Oestrogen causes endometrial prolife­ ration; progesterone causes endometrial maturation; shedding follows withdrawal of oestrogen and progesterone. If oestrogen is given alone, the endometrium proliferates uncontrolled, resulting in irregular and heavy bleeding, polyps, and i risk of endometrial carcinoma. Caused by anovulatory cycles or administration of unopposed oestrogen. Endometrial cancer  In the UK, ~7,700 women each year are diagnosed with endometrial cancer (5% of all 5 cancers). It is predominantly a disease of postmenopausal women with 93% of cases diagnosed in women >50y (peak age 70–74y). Risk factors: • Drugs—unopposed oestrogen, tamoxifen • Age • Granulosa cell ovarian tumour • Obesity • FH—of breast, ovary, or colon cancer • Nulliparity • Previous pelvic irradiation • Late menopause • Hereditary non-polyposis colorectal cancer • DM Risk is d with current or past use of the COC pill and/or progestogens. Presentation  15% are asymptomatic. Most present with PMB (75–80%)— any woman presenting with PMB has endometrial carcinoma until proven otherwise. Premenopausally tends to occur in overweight women and present with continual bleeding. Rarely detected on cervicalsmear. Management  Refer any woman with PMB to gynaecology for further investigation. Assessment comprises transvagin*l USS to look at endometrial thickness ± endometrial sampling with pipelle or hysteroscopy. Treatment  TAH and BSO ± radiotherapy and/or chemotherapy depending on the likelihood of recurrence. Survival depends on the age of the patient and stage/grade of the tumour. Stage Idisease—85% 5y survival; stage IV—25% 5y survival. Endometritis Acute infection of the endometrium. Uncommon amongst premenopausal women. Usually occurs after surgery (including IUCD insertion) or childbirth. Presents with fever, lower abdominal pain, uterine tenderness, and/or purulent discharge (may be blood stained). Take high vagin*l/endocervical swabs for M,C&S (including chlamydia). Management  Treat with antibiotics, e.g. doxycycline 100mg bd for 14d + metronidazole 400mg bd for 1wk or azithromycin 1g stat for chlamydia. Pyometra is a complication (uterine cavity fills with pus)—suspect if endometritis fails to clear and refer to gynaecology urgently. Further information NICE Referral guidelines for suspected cancer (2005) www.nice.org.uk

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Ovarian disease Refer urgently to gynaecology (to be seen in 50y) if a woman reportsN • New IBS>50y • Unexplained weight d, fatigue, or change in bowelhabit • Any of the following >12x/mo: • Feeling full (‘early satiety’) and/ • Persistent abdominal or loss of appetite distension (‘bloating’) • i urinary frequency/urgency • Pelvic/abdominalpain If CA125 is ≥35iu/mL  refer for USS. If suspected ovarian cancer on USS refer urgently to gynaecology (to be seen in8cm diameter; or 15cm diameter at diagnosis

15%

Mucinous Can be very large; often multilocular; often contain viscid mucin—if burst, can cause pseudomyxoma peritonei (mucin -secreting cells are spread throughout the peritoneum)

Benign mucinous cystadenoma—peak incidence aged 30–50y; 80% of mucinous tumours; bilateral in 5–10%; 20–25% of all benign ovarian tumours

100%

Borderline mucinous cystadenoma—10% of mucinous tumours; bilateral in 10%

90–95%

Malignant mucinous cystadenocarcinoma— Peak age 40–70y; 10% of mucinous tumours; bilateral in 15–30%; 5–10% of all p ovarian cancers; average diameter at diagnosis 16cm

34%

Endometrioid—Peak age 50–60y; 30–50% bilateral; benign tumours are rare; malignant tumours account for 20–25% of all malignant ovarian neoplasms; 30% coexist with endometrial cancer; 10% coexist with endometriosis Clear cell (mesonephroid)—5% bilateral; 5–10% of all malignant ovarian neoplasms; 25% coexist with endometriosis; associated with hypercalcaemia Brenner (transitional cell)—Rare—2–3% of all ovarian tumours; >90% are benign. If malignant, have poor prognosis; 10% patients receiving gonadotrophin therapy. Abdominal pain/ swelling ± vomiting/diarrhoea. Manage with rest and simple analgesia, e.g. ibuprofen or paracetamolprn. • Severe 1% patients receiving gonadotrophin therapy. Abdominal pain/ distension, vomiting/diarrhoea, ascites, pleural effusion, and/or venous thrombosis.Admit. Further information NICE Ovarian cancer (2011) M www.nice.org.uk

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Ovarian cancer and polycystic ovaries Refer urgently to gynaecology (to be seen in 50y) if a woman reportsN • New IBS>50y • Unexplained weight d, fatigue, or change in bowelhabit • Any of the following >12x/mo: • Feeling full (‘early satiety’) and/ • Persistent abdominal or loss of appetite distension (‘bloating’) • i urinary frequency/urgency • Pelvic/abdominalpain If CA125 is ≥35iu/mL Refer for USS. If suspected ovarian cancer on USS, refer urgently to gynaecology (to be seen in12 cysts of 2–9mm in diameter (string of pearlssign) • Blood i testosterone (>2.5nmol/L—if >4.8nmol/L, exclude other causes of androgen hypersecretion, e.g. tumour, Cushing’s syndrome), d sex hormone binding globulin (SHBG) Complications  Insulin resistance—2x i incidence of DM; i CVD risk— central body fat distribution, obesity, i BP, i triglycerides, d HDL; 3x i risk of stroke/TIA; i endometrial cancer risk; obstructive sleep apnoea. Management  In all cases, encourage weight d and exercise. • If oligomenorrhoeic consider progestogens to induce a withdrawal bleed every 2–3mo to d risk of endometrial hyperplasia • Consider the COC pill to regulate menstruation; COC pills with anti-androgen (e.g. co-cyprindiol) may d acne/hirsutism • Consider offering annual HbA1c/FBG check—particularly if obese (BMI >30), FH of DM, or aged >40y. Screen pregnant women with PCOS for gestational DM with a GTT at 4.5 and typical clinical picture.

ManagementG  Without treatment, 50% remit spontaneously. Cure rate with all methods is 785%. There is no benefit from treating the woman’s partner. Treatment: • Metronidazole 400mg bd for 5–7d or 2g singledose • Clindamycin 2% cream 5g nocte pv for1wk • Recurrent infection—X suppressive therapy using metronidazole 400mg bd for 6d to cover each period, metronidazole 0.75% gel 2x/ wk pv for 4–6mo, lactic acid gel alternate nights for >1mo, and pv probiotics are all but robust evidence of effectiveness is lacking

Candidiasis (thrush)  Fungal infection—~20% of patients are asymptomatic. Predisposing factors include: • Cushing’s or Addison’s disease • Immunosuppression • DM • Pregnancy • Steroid treatment • Broad-spectrum antibiotics • vagin*l trauma • Radiotherapy/chemotherapy Presentation  Well, pruritus vulvae, superficial dyspareunia, and/or thick, creamy, non-offensive discharge. Examination: discharge (cottage cheese) and sore vulva which may be cracked/fissured. Investigation is usually unnecessary. Confirm diagnosis if infection persists or recurs by sending a swab from the anterior fornix forM,C&S. ManagementG  Only treat if symptomatic. Sexual transmission is minimal; there is no benefit from treating the partner unless overt infection: • Try clotrimazole pessaries—cure rate~90% • Alternative is oral fluconazole 150mg stat, repeated after 3d if severe infection. Contraindicated in pregnancy or lactation—83% curerate Recurrent infectionG  Advise loose, cotton underwear and avoidance of soaps, perfumes, or disinfectants in the bath. Consider vulval emollients to treat associated dermatitis. If �4 documented episodes (�2 confirmed with microbiology) in a year, treat with fluconazole 150mg every 3d x3, then 150mg weekly for6mo. Further information FSRH Management of vagin*l discharge in non-genito-urinary medicine settings (2012) M www.fsrh.org BASHH M www.bashh.org • Management of bacterial vaginosis (2012) • Management of vulvovagin*l candidiasis (2007)

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Sexually transmitted infection GPs are frequently presented with symptoms/signs either presented directly or found incidentally (e.g. when doing a cervical smear) that may indicate sexually transmitted infection (STI). The easiest (and often best) option is to refer suspected cases to a genito-urinary medicine (GUM) clinic. Sometimes the patient is reluctant to go and 40% referrals never attend, so it is still necessary for GPs to know how to prevent, diagnose, and treat STI themselves.

Contact tracing  Best done by GUM clinics. If a patient refuses to go,

then provide him/her with a letter to give to contacts stating the disease he/she has been in contact with, treatment given, and suggesting contacts visit their local GUM clinic promptly.

Use of GUM clinics  In general, refer patients: • Who require contact tracing • If counselling is needed, e.g. first attack of HSV,HIV • If diagnosis is still unclear after investigation • For confirmation of diagnosis, e.g.HSV • If specialist treatment is required, e.g. treatment of genitalwarts Prevention of STIs  NICE recommends: • Identification of high-risk patients opportunistically in general practice, e.g. at new patient checks, when attending for travel advice • One-to-one discussions with those at i risk of STIs lasting 15–20min, structured on the basis of behaviour change theories to d risk-taking High-risk groups Include patients with STIs; men who have had sex with other men; people who have come from/visited areas of high HIV prevalence; substance/alcohol misuse; early onset of sexual activity; unprotected sex; and frequent change of/multiple partners. Young people from vulnerable groups (e.g. from disadvantaged backgrounds; in/leaving local authority care; low educational attainment). Should be offered one-to-one sessions aimed at educating them about sexual health and contraception. Chlamydia screening A screening programme for the under 25s is currently in operation in the UK (b p.740). Vaccination  A vaccine targeting the most common forms of HPV causing cervical cancer is now available in the UK. The target population is girls aged 12–14y before they become sexually active (b p.749), but potentially any sexually active woman could benefit. Prevention of HIV  bp.744 vagin*l discharge  bp.736 Acute pelvic inflammatory disease (PID)  May be asymptomatic. Peak age 15–25y. >10% develop tubal infertility after 1 episode; 50% after 3 episodes. Risk of ectopic pregnancy i x10 after a single episode. Only 70% of those with acute PID clinically have diagnosis confirmed on laparoscopy. Most cases of PID are associated with STI—usually chlamydia (50%) or gonorrhoea. In 20% no cause isfound.

Sexually transmitted infection

History • Abnormal vagin*l bleeding— • Fever >38oC and malaise heavier periods, intermenstrual • Acute pelvic pain (usually and/or post-coital bleeding bilateral) and deep dyspareunia • Purulent vagin*l discharge • Dysuria Examination  Pyrexia, bilateral lower abdominal tenderness, vagin*l discharge, cervical excitation, and adnexal tenderness. Investigations  Consider swabs (HVS and endocervical swab for M,C&S and chlamydia/gonorrhoea screen) and blood tests (FBC—may show leucocytosis; i ESR/CRP). ManagementG  Admit if very unwell, pregnant, or if ectopic pregnancy or other acute surgical emergency cannot be excluded. Otherwise: • Advise rest and sexual abstinence; provide analgesia • Treat with ofloxacin 400mg bd and metronidazole 400mg bd for 14d; alternative is ceftriaxone 500mg IM as a single dose, followed by oral doxycycline 100mg bd and oral metronidazole 400mg bd for14d • If the patient has an IUCD consider removal, but only if symptoms are severe. If removed advise re alternative contraception and emergency contraception if sexual intercourse 1 STI simultaneously.

Chlamydia  Major cause of pelvic pain and infertility inwomen. Screening  Chlamydia is a preventable cause of infertility, ectopic pregnancy, and pelvic inflammatory disease. Screening using urine testing or self-taken swabs d prevalence and incidence of pelvic inflammatory diseaseS. Screening programmes aimed at young people 70% are asymptomatic. Symptoms: vagin*l discharge (30%); post-coital or intermenstrual bleeding; pelvic inflammatory disease (10–30%—b p.738); dysuria • Examination Mucopurulent cervicitis; hyperaemia and oedema of the cervix ± contact bleeding; tender adnexae; cervical excitation • Investigation Send endocervical (if symptoms) or self-taken vulvovagin*l swab (if asymptomatic) for NAAT to confirm diagnosis 0 If 4 or 5 and exposed to potential infection 2wk after exposure. Management • Doxycycline 100mg bd for 1wk or erythromycin 500mg qds for 14d; azithromycin 1g stat po is an alternative which ensures compliance • During pregnancy/breastfeeding use erythromycin 500mg qdsfor 2wk • Consider supplying home-testing kits to patients at high risk ofSTIs Presentation in neonates Conjunctivitis, pneumonia, otitis media, pharyngitis—1 in 3 affected mothers have affected babies. Seek specialist advice if suspected.

Gonorrhoea  Transmission: 4—1 in 5 exposures; 5—1 in 2 exposures. Presentation in men  Depends on site of infection: • Urethral infection (90% asymptomatic) If asymptomatic—send first-catch urine sample for NAAT; if symptomatic send urethral ± rectal ± pharyngeal swabs (as appropriate) for NAAT and M,C&S to confirm diagnosis. Presentation in women Infection may be asymptomatic (50%) or cause vagin*l discharge (50%), lower abdominal pain; dysuria but not frequency (25%); abnormal vagin*l bleeding; pelvic inflammatory disease; abscess of

Chlamydia, gonorrhoea, and trichom*onas

Bartholin’s gland; miscarriage; or preterm labour. Rectal and pharyngeal infections are usually asymptomatic. • If asymptomatic—send self-taken vulvovagin*l swab forNAAT • If symptomatic—send endocervical swab ± rectal ± pharyngeal swab (as appropriate) for NAAT and M,C&S to confirm diagnosis ManagementG • Ceftriaxone 500mg IM as a single dose + azithromycin 1g statpo • Refer to GUM for contact tracing • If persisting symptoms/signs after treatment, test for cure with swabs sent for M,C&S >72h after completion of therapy • If asymptomatic following treatment, test for cure 2wk after treatment completion with NAAT, followed by culture if NAAT-positive • If persistent infection, seek specialist advice

Trichom*onas vagin*lis(TV) Presentation in neonates Ophthalmia neonatorum. Purulent discharge from the eyes of an infant 4.5, TV is a possibility; send HVS from the posterior fornix at the time of speculum examination for M,C&S. Sensitivity of HVS is low for TV, so if HVS is–ve, consider GUM referral for wet microscopy ± culture and contact tracing. 0 TV may rarely be detected on cervicalsmear. ManagementG • Advise patients to avoid sexual intercounse until they and their partner(s) have completed treatment and follow-up • Metronidazole po (400mg bd for 5–7d or 2g stat)—tinidazole 2g stat is an alternative • Consider referral to GUM clinic for contact tracing • Resistant TV—try higher dose metronidazole:400mg tds po + 1g od pr for 7d, or metronidazole 2g od for 3–5d. High-dose tinidazole 2g bd for 2wk ± topical vagin*l tinidazole is an alternative

Further information BASHH M www.bashh.org • Management of Chlamydia trachomatis genital tract infection (2006) • UK national guideline on gonorrhoea testing (2012) • Management of gonorrhoea in adults (2011) • Management of Trichom*onas vagin*lis infection (2007) National Chlamydia Screening Programme (NCSP) M www.chlamydiascreening.nhs.uk

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Hepatitis BandC Hepatitis B (HBV)  Common. Endemic in much of Asia and the Far East. The virus has 3 major structural antigens:surface antigen (HBsAg), core antigen (HBcAg), and e antigen (HBeAg). Spread is via infected blood, sexual intercourse, from mother to newborn baby, or via human bites. Incubation period is 6–23wk (average17wk). High-risk groups  Patients who are/have: • Injecting drugusers • At risk due to occupation e.g. • Many sexual partners healthcare workers • Adopted children from high/ • Staff/residents of residential accommodation for individuals intermediate-risk countries • Foster parents with mental handicap • Close family contacts of a case/ • Travelling to high/intermediatecarrier riskareas • Receiving regular blood/blood • Babies born to mothers who are chronic carriers of hepatitisB products and their carers • Chronic renal/liver disease or have had acute hepatitis B in • Prison inmates pregnancy Presentation  May be asymptomatic or present with fever, malaise, fatigue, arthralgia, urticaria, pale stools, dark urine, and/or jaundice. Investigation  LFTs (hepatic jaundice—i bilirubin, i ALT/AST, i alkaline phosphatase), hepatitis serology (see Figure22.1). • HBsAg Present from 1–6mo post-exposure. If present >6mo after the acute episode defines carrier status • HBeAg Present from 6wk–3mo after acute illness. Indicates high infectivity • Anti-HBs Antibodies appear >10mo after infection; imply immunity Management  In all cases advise patients to avoid alcohol. Refer for specialist advice. Treatment is supportive for acute illness. Chronic hepatitis is treated with interferon and lamivudine with varying success. Prognosis  785% recover fully; 10% develop carrier status; 5–10% develop chronic hepatitis—may lead to cirrhosis and/or liver carcinoma. Fulminant hepatitis and death are rare(3h late (>12h late for desogestrel POP) and unprotected sexual intercourse has occurred in the 2d followingthis. Diarrhoea/vomiting  Continue taking the POP but use an additional barrier method during the episode and for 2d afterwards. Interactions with other drugs Efficacy of POPs is not affected by antibacterials that do not induce liver enzymes. Efficacy is d by enzyme-inducing drugs (b p.756)—advise women to use an additional barrier method or alternative contraceptive method during treatment and for >4wk afterwards. Advise an alternative method of contraception if taking long-term hepatic-enzyme-inducingdrugs. Follow-up  Review 3mo after starting the POP or changing from CHC— earlier if complications. Once established, review every 6–12mo—assess risk factors and side effects; give health education, e.g. smoking cessation advice, information about STIs, information about long-acting reversible contraception; checkBP.

Injectable progestogens  (BNF 7.3.2.2) Useful if oestrogen-containing

preparations are contraindicated or compliance is a problem. Failure rate is 6wk after childbirth—if not breastfeeding, first dose can be given 12wk and 5d—see Table22.4 • CSM advice about Depo-Provera® • In all women, weigh benefits of use for >2y againstrisks • In women with risk factors for osteoporosis, consider a method of contraception other than Depo-Provera® • In adolescents, Depo-Provera® should only be used only when other methods of contraception are inappropriate Noristerat®  Norethisterone enantate 200mg/mL. Warm first then give 1× 1mL by deep IM injection into the gluteal muscle before day 6 of the cycle or immediately after childbirth (avoid breastfeeding if baby has jaundice requiring treatment). Do not rub the injection site afterwards. May be repeated once only after 8wk. Unlicensed if repeated further. Interactions  Effectiveness is not d by antibacterials that do not induce liver enzymes. Effectiveness of Noristerat® (but not Depo-Provera®) is d by enzyme-inducing drugs—advise additional contraception whilst taking these drugs and for 4wk after stopping or alternative method.

Progestogen implant  (see BNF 7.3.2.2) Nexplanon® is the only implant currently available in the UK. It is a radio-opaque flexible rod (40mm × 2mm) containing 68mg of etonogestrel that is inserted subdermally into the lower surface of the upper arm on day 1–5 of the cycle. If inserted after day 5, check not pregnant and use an additional method for7d. Advantages  Lasts 3y and, once inserted, no compliance required; can be used for women at risk of ectopic pregnancy; no effect on bone density; once removed, fertility returns immediately to normal. Disadvantages  A minor operation is needed for insertion/removal. Special training is needed, and complications of minor surgery can occur (e.g. infection, scarring). d efficacy with liver enzyme-inducing drugs— advise additional method for duration of treatment and 4wk afterwards or alternative contraception if enzyme-inducing drugs are being used

Progestogen-only contraceptives

long-term. Cannot be used as part of a HRT regime. May cause menstrual disturbances—exclude other causes. Treat with oestrogen (Marvelon® contains the same progestogen), additional progestogen, or NSAID. Other side effects include acne, mood swings, breast tenderness, change in libido—treat symptoms as needed. Table22.4  Late Depo-Provera® guidelines Timing of DepoProvera®

Has unprotected sex occurred?

Up to 12wk N/A and 5d since date of previous injection No

When an injection is overdue

Can the Is emergency injection be contraception given? needed?

Are condoms or abstinence advised?

Should a pregnancy test be done?

Yes

No

No

No

Yes

No

Yes—for the No next 14d*

Yes—but only in Yes—or give Yes the last 3d desogestrel 75 micrograms for 21d

Yes—for the Yes—21d next 14d* later

Yes—but only in Yes—or give Yes—offer the last 3–5d desogestrel copper IUD 75 micrograms for 21d

No

Yes—21d later

Yes—>5d ago

Yes—for 21d until a pregnancy test is confirmed negative and for a further 14d* after giving Depo injection

Yes—at initial presentation and 21d later

No

No

X *WHO/FSRH recommendations state that injections of Depo-Provera® can be given up to 14wk and Noristerat® can be given up to 10wk after the previous injection without the need for additional barrier contraception. These guidelines also state that, when needed, additional contraception is only necessary for7d.

Further information NICE Long-acting reversible contraception (2005) M www.nice.org.uk FSRH M www.fsrh.org • Progestogen-only pills (2009) • Progestogen-only injectable contraception (2009) • Progestogen-only implants (2008) • Nexplanon® (2010)

Patient information Family Planning Association (FPA) F 0845 122 8690 (0845 122 8687 in Northern Ireland) M www.fpa.org.uk

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Intrauterine devices Intrauterine contraceptive device (IUCD) (BNF 7.3.4) Plastic

carrier wound with copper wire/fitted with copper bands. Suitablefor: • Older parouswomen • As second-line contraception in young nulliparous women,or • For emergency contraception Acts by inhibiting fertilization, sperm penetration of the cervical mucus, and implantation. Pregnancy rate with IUCDs containing 380mm2 copper is 7cm

Mini TT 380 Slimline®

5y

>5cm

Multiload Cu375®

5y

6–9cm

Multi-Safe 375®

5y

6–9cm

Nova-T 380®

5y

6.5–9cm

Neo-SafeT380®

5y

6.5–9cm

T-Safe Cu380A Quickload®

10y

6.5–9cm

TT 380 Slimline®

10y

6.5–9cm

UT 380 Short or Standard®

5y

Short—5–7cm Standard—6.5–9cm

Mirena®

5y*

>6.5cm

*4y if being used for prevention of endometrial hyperplasia

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IUCD andIUS • Fitting and removal Requires specialist training and can be uncomfortable for thewoman • Expulsion/malposition—Risk of expulsion is ~1 in 20. Usually occurs 30iu/L stop contraception after 1y of amenorrhoea; if �50y and not amenorrhoeic, consider investigating abnormal bleeding and continue >55y until amenorrhoeic for1y

Further information Family Planning Association (FPA) F 0845 122 8690 (0845 122 8687 in Northern Ireland) M www.fpa.org.uk FSRH M www.fsrh.org • Contraception for women aged over 40years (2010) • Contraceptive choices for young people (2010) • UK Medical Eligibility Criteria for contraceptive use (2009)

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Termination of pregnancy The role oftheGP • The earlier in pregnancy a termination of pregnancy is performed, the lower the risk of complications. General practice is often the first stage of the referral procedure—have arrangements which minimizedelay • Termination of pregnancy (TOP), especially for ‘social’ reasons, is a difficult ethical area for many GPs. Whatever your views, be sympathetic and if not prepared to refer yourself, arrange for the patient to see someone who will do so as soon as possible • Confirm pregnancy if unsure. Assess dates by bimanual palpation or arrange datingUSS • Counselling—unbiased counselling to allow a woman to reach a decision she feels is right for her—this is an important decision she will have to live with for the rest of her life. Why does she want a termination? Has she considered alternatives? Does her partner/do her parents know? What are theirviews? • Ideally the woman should be given some time once she has all the information to make her decision (e.g. follow-up in a few days). Offer a let-out clause—she can always change her mind right up until the time of the procedure, and you will support her whatever decision shemakes • Consider signing form HSA1 (remember to include your qualifications) • Discuss contraception after TOP (ideally do this before TOP so it can be started immediately after) • Arrange follow-up after the procedure

Legal constraints  The 1967 and 1990 Human Fertilization/Embryology Acts govern termination of pregnancy in the UK. Termination is allowed at 24wk can only be carried out in NHS hospitals. 99% TOPs take place 20wk are usually performed when fetal abnormality is found on USS (or amniocentesis) or if pregnancy is concealed in the veryyoung. 0 Seek medicolegal advice from your medical indemnity organization if the patient is 35y. Female • Rubella status • Chlamydia serology—indicator of possible tubal disease • Mid-luteal progesterone—check on day 21 of the menstrual cycle for a woman with a 28d cycle; adjust timing if longer/shorter cycle. Can only be accurately interpreted after the next period as aims to ‘catch’ the progesterone peak 7d before the next period. Normal value (>30nmol/L) signifies ovulation • FSH/LH—check on day 1–5 of the menstrualcycle • Consider TFTs if symptoms/signs of thyroid disease, or prolactin if galactorrhoea or any suggestion of pituitary tumour Male  Sperm problems affect 71 in 5 couples. sem*n analysis is important even if the man already has children. If the first test is abnormal, advise loose trousers and underwear and repeat after 3mo—or as soon as possible if grossly abnormal. 0 Abnormal sperm do not fertilizeova. Instructions for producing a sem*n sample for analysis No sex for 2d beforehand and �7d since last sex (may affect motility). masturbat* into labelled sterile pot without use of condoms/gels. Keep the sample warm (e.g. inside pocket), and deliver to the laboratory within 2h. Handover directly to a member of laboratory staff if possible. Referral  Local protocols vary and exclusions may apply. Generally refer after 18mo of failure to conceive despite regular intercourse. Refer sooner if abnormal history, examination, or investigations,e.g.: • Female age >35y; amenorrhoea/oligomenorrhoea; PCOS; previous pelvic inflammatory disease orSTI • Male Previous genital pathology or urogenital surgery; varicocele; significant systemic illness; persistent abnormality on sem*n analysis

Possible treatments GPs may need to continue to prescribe • Clomifene—ovarian stimulation, treatment for oligospermia • Tamoxifen—may be prescribed to women intolerant of clomifene • Metformin—used as an adjunct to clomifene in overweight ladies with polycystic ovarian syndrome who fail to respond to clomifenealone • Others, e.g. gonadotrophins, dopamine agonists (e.g. bromocriptine) Counselling  Consider early referral for specialist counselling. Access is through national support groups and at local specialist fertility centres. Useful contacts: • British Infertility Counselling Association M www.bica.net • British Fertility Society M www.britishfertilitysociety.org.uk

Further information NICE Fertility:Assessment and treatment of people with fertility problems (2004) M www.nice.org.uk

Advice and information for patients Infertilitynetwork UK F 0800 008 7464 M www.infertilitynetworkuk.com

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Sexual problems Sexual problems may have a physical or psychological basis but all develop a psychological aspect in time. Both partners have a problem in 730% cases. Be supportive—your response will determine whether the patient receives appropriatehelp.

Assessment • History of the problem What is the problem? If new, when did it start? Why consult now? What outcome does the patient want? Is the patient complaining or is his/her partner? • Sexual history Details of sex education; attitude towards sex; past history of sexual problems (or lack of problems) • Medical history Chronic disease; psychiatric problems; current medication • Social history and recent life events • Examination Genitalia for abnormalities or tenderness—helpful but do not insist as it may scare the patientaway 0 Always consider psychological aspects Poor self-image; anger or resentment—relationship/financial difficulties, children, parents, work stress; ignorance or misunderstanding; shame, embarrassment, or guilt— view that sexuality is ‘bad’, sexual abuse; anxiety/fear about sex—fear of closeness, vulnerability, letting go, and failure. Lack of sexual interest  Usually needs specialist help. Often there are underlying psychological difficulties which may relate specifically to sex, e.g. previous child abuse, or a general psychological disorder. Women frequently lose interest around the menopause or after operations (especially mastectomy or hysterectomy) or if their partner’s performance repeatedly leads to frustration (e.g. impotence). Both sexes lose interest if depressed or after traumatic events.

Vaginismus  Usually apparent at vagin*l examination—severe spasm of the vagin*l muscles and adduction of thighs. May be detected incidentally when undertaking routine procedures, e.g. cervical smear. Try to find the root cause. Common causes: • Fear of the unknown • Localpain • Past history of rape, abuse, or severe emotional trauma • Defence mechanism against growingup Management  Treat any underlying medical disorder causing pain. Desensitize by encouraging the woman to examine herself, and also encourage the partner to be confident enough to insert a finger into the vagin*. If no success,refer.

org*smic problems in women  Consider: Physical reasons • Drugs—major tranquillizers, antidepressants • Neurological disease • Pelvic surgery—recognized complication of hysterectomy

Sexual problems

Psychological reasons • Women who have never achieved an org*sm May have psychological reasons. Give ‘permission’ for the woman to investigate her body’s own responses further by masturbation or vibrator. When she has learned how to relax, encourage her to tell her partner and incorporate caressing into their usual lovemaking • Women who have lost the ability to achieve org*sm May need specialist help, especially about current relationship or loss of self-image Dyspareunia  b p. 714    Erectile dysfunction  bp.776

Premature ejacul*tion  ejacul*tion sooner than either partner wishes. With practice men can learn to delay ejacul*tion. The stop/start technique may be effective:when during caressing or intercourse, a man feels he is close to climax he should stop being stimulated and relax for 30 seconds; stimulation can then recommence until he is close to climax again, when the relaxation is repeated. If this fails, the woman should squeeze the penis at the base of the glans between finger and thumb during relaxation phases. Consider referral for sex therapy if no improvement. Delayed ejacul*tion  May be a sign of long-standing sexual inhibition. Often patients can ejacul*te by masturbation but not intravagin*lly. Explore anxiety and guilt feelings. Use a strategy like that for psychogenic erectile dysfunction (b p.778). If that fails, refer for psychosexual counselling. Retrograde ejacul*tion  sem*n passes into the bladder rather than the urethra—complication of TURP or bladder neck incision. May also occur as a result of spinal injury or DM. The patient can usually achieve an org*sm but there is no ejacul*te or the volume of the ejacul*te is d. Urine may be cloudy after having sex. Confirm diagnosis with urine microscopy (excess sperm in urine). Unless infertility is a problem, no treatment is required. Haematospermia  Blood in the ejacul*te. Common causes include urogenital infection and minor urethral trauma, but often no cause is found. If persistent, underlying pathology is more likely. Ask about other symptoms, e.g. discharge, pain, dysuria. Examine the external genitalia and perform DRE to assess the prostate. Check MSU and sem*n analysis ± urethral swab (including chlamydia) if any urethral discharge/high risk of STI. Check PSA and urine cytology if patient is aged >40y. If persists and no cause is found, refer to urology. Further information about specialist doctors/therapists College of Sexual and Relationship Therapists F 020 8543 2707 M www.cosrt.org.uk Institute of Psychosexual Medicine M www.ipm.org.uk

Information for patients Brown P, Faulder C (1989) Treat Yourself to Sex. Harmondsworth: Penguin. ISBN:0140110186.

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Erectile dysfunction 50% men aged 40–70y experience inability to obtain/maintain sufficient rigidity of the penis to allow satisfactory sexual performance; 90% are too embarrassed to seek help—always ask. Incidence i withage. Organic causes(80%) • Cardiovascular CHD i incidence x4—more likely to have multi-vessel than single-vessel coronary artery disease; peripheral vascular disease; hypertension—incidenceix2 • DM incidence i ×3. >35% of diabetic men have erectile dysfunction. May be the presenting featureofDM • Neurological, e.g. pelvic surgery, spinal injury, multiple sclerosis • Side effects of prescription drugs Consider changing medication if onset of erectile dysfunction is within 2–4wk of initiation of drug therapy e.g. thiazides • Smoking (incidence i x2), alcohol, or drugabuse • Peyronie’s disease (b p.464) • Testosterone deficiency or hyperprolactinaemia

Psychogeniccauses • Performance anxiety • Depression or stress

• Relationship failure • Fear of intimacy

Drugs causing erectile dysfunction

• Antihypertensives • Major tranquillizers • Antidepressants (e.g. SSRIs) • Anti-androgens

• Finasteride • Cimetidine

History  Ensure the presenting problem is erectile dysfunction and not other sexual difficulties; identify risk factors and distinguish psychogenic from organic causes (see Table 22.6). 0 Many with organic erectile dysfunction develop a psychogenic component which perpetuates symptoms. Examination and investigation • CVD and DM—check BP, peripheral pulses, and blood for fasting lipid profile and glucose • Psychological distress—consider depression/anxiety screening • Testosterone insufficiency—genitals (small/absent), breasts i, d beard (d frequency of shaving). If suspected, check serum testosterone, sex hormone binding globulin, free androgen index, FSH/LH ± prolactin Table22.6  Is erectile dysfunction organic or psychogenic? Onset sudden or gradual?

Psychogenic origin

Organic origin

Sudden onset

Gradual onset

Consistent loss of erections?

Inconsistent response

Consistent failure

Does the patient ever wake up with an erection?

Early-morning erections

Loss of early-morning erections

Does the patient want to have intercourse?

Relationship problems

Normal libido

Age?

Usually 60y

Erectile dysfunction

Table22.7  Treatment options for erectile dysfunction Treatment

Notes

Oral drugs Phosphodiesterase Effective for 70%. Use prn before intercourse (see Table22.8). Only use 1x/d. Avoid if patient has unstable type 5 inhibitors, angina, recent stroke, or MI. Do not give a nitrate within e.g. sildenafilS 24h of use Apomorphine

2–3mg prn 20min before sexual activity

YohimbineS

Herbal remedy available OTC. 10–30mg od is effective May cause insomnia

Local drug treatments Intraurethral or intracavernosal alprostadil

Intraurethral preparation is effective for 40% and intracavernosal preparation for 80% patients. Used prn. Requires some manual dexterity. Takes ~10min to work. Penile pain is common. Prolonged erection and priapism results in ~1%. Advise patients to seek medical help if erection >4h

Mechanical devices Vacuum devices

80% effective. The penis is placed in the device and air withdrawn mechanically sucking blood into the penis. Erection is maintained by placing a constriction band around the base of the penis

Penile prosthesis

Last resort. Inflatable or rigid. Major complication is infection

Others Androgen supplements

Ineffective unless documented hypogonadism. Only use with specialist advice. Exclude prostatic cancer and significant CVD first, and check PSA and haematocrit at 3,6 and 12mo after initiation of treatment, then annually thereafter

Psychotherapy

Effective for some. Time-consuming and expensive but may avert the need for drugs and give permanent resolution

X Andropause/male menopause From 730y, testosterone levels d by 710% every decade. At the same time, sex hormone binding globulin (SHBG) level i, which d the amount of bioavailable testosterone further. Andropause is associated with low bioavailable testosterone levels; 730% of men in their 50s develop symptoms. Presentation  d sex drive, emotional, psychological, and behavioural changes, d muscle mass and muscle strength, i upper and central body fat, osteoporosis and back pain, i cardiovascularrisk. Management  If suspected, check total testosterone and SHBG ± FSH/ LH and prolactin. If hypogonadism is confirmed, refer for specialist management with testosterone replacement.

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GP management  Counsel the couple about the problem, its possible causes and management (see Table 22.7, b p.777, and Figure22.4). • Advice on lifestyle—d smoking and alcohol. Weight loss and iexercise for obese, underactive patients improves both sexual function and cardiovascular health • Discuss pros and cons of available drug treatment. Phosphodiesterase type 5 inhibitors (PDE5s) are the mainstays of treatment—titrate dose to effect (most people with DM need the maximum dose); warn the patient he may need 8 attempts before a satisfactory erection occurs; side effects include headache, flushing, and acid reflux • Review progress—adjust dosage, consider other treatment options (intraurethral/intracavernosal alprostadil, vacuum devices) or treatment for psychosexual problems, and/or referral Referral  Options: • Urologist If the patient has never had an erection, has a severe vascular problem, lack of success with treatment in general practice, or severe psychological distress due to erectile dysfunction • Endocrinologist Hormone abnormalities (e.g. d androgen, iprolactin)—treatment does not always restore potency • Psychiatrist/psychosexual counsellor Age 25y with erectile dysfunction should be screened for DM, cardiac risk factors, and signs/symptoms of vascular disease. 0 NHS prescriptions for erectile dysfunction are available only formen: • Treated for prostate cancer; with kidney failure, spinal cord injury, DM, MS, spina bifida, Parkinson’s disease, polio, severe pelvic injury, or who have had radical pelvic surgery or a prostatectomy • Already receiving drug treatment for impotence on 14.9.98 • Through specialist services for men suffering severe distress due to erectile dysfunction Endorse NHS prescriptions with the letters ‘SLS’. A consultation is currently underway to lift NHS prescribing restrictions.

Further information British Society for Sexual Medicine Guidelines for the management of erectile dysfunction (2007) M www.bssm.org.uk

Erectile dysfunction

Erectile dysfunction Check for recent drug therapy, e.g. thiazides

Check for cardiovascular disease

History and examination

Check for medical conditions, e.g. DM, hyperlipidaemia, i BP; consider endocrine and urological disorders Patients not on nicorandil or nitrates

Manage medical conditions

Substitute (if possible) nitrates/nicorandil for alternative treatment, e.g. amlodipine

Patients on nicorandil and/or nitrates

Treat with oral therapy —Sildenafil —Tadalafil —Vardenafil —Apomorphine* Non-responders to maximum dose Refer to clinic

Discuss other treatment options

* Not as effective as PDE5 inhibitors but not contraindicated with nitrates.

Figure22.4  Algorithm for management of erectile dysfunction Table22.8  PDE5 inhibitors and actiontimes Drug

Onset of action in min (peak action)

Duration of action in h

Doses

Sildenafil

20–30 (60)

4–6

25–50–100mg

Tadalafil

60–120 (120)

36–48

10–20mg*

Vardenafil

20–30 (60)

4–6

5–10–20mg

*  For patients who anticipate sexual activity �2x/wk, 2.5–5mg od can be used instead. Figure 22.4 and Table 22.8 are reproduced from British Heart Foundation Factfile:Drugs for erectile dysfunction (6/2005) available from M www.bhf.org.uk

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Pregnancy Pre-conception and early pregnancy counselling  782 Antenatal care  786 Health promotion for pregnant women  792 Who should deliver where?  794 Screening in pregnancy  796 Common symptoms in pregnancy  802 Pruritus and rashes in pregnancy  804 Rubella and parvovirus in pregnancy  806 Other rash illnesses in pregnancy  808 Other infections in pregnancy  810 Bleeding in early pregnancy  814 Ante- and postpartum haemorrhage  818 Haemolytic disease and rhesus isoimmunization  820 A–Z of medical conditions in pregnancy  822 Hypertension in pregnancy  826 Diabetes and epilepsy in pregnancy  828 Intrauterine growth and malpresentation  830 Labour  832 Maternal postnatal care  836 Common postnatal problems  838 Stillbirth and neonatal death  842

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Pre-conception and early pregnancy counselling The aim of pre-pregnancy care is to give a woman enough information for her pregnancy to occur under the optimal possible circ*mstances. Areas to coverare: Smoking  d ovulation, d sperm count, d sperm motility. Once the woman is pregnant  Smoking: • i miscarriage rate (x2) and risk of ectopic pregnancy • i risk of placenta praevia and placental abruption • i risk of premature rupture of membranes and preterm delivery • i risk of cleft deformities • i perinatal mortality and d birth weight (by an average of ~200g) Once the baby has delivered  Smoking is associatedwith: • i rate of cotdeath • i chest infections and otitis media in children 27% of pregnant women are smoking at the time of delivery. Explain risks and advise on ways to stop—b p.182. Alcohol  Fetal alcohol syndrome (growth restriction, CNS involvement, and facial deformity) is rare and tends to occur in babies of heavy drinkers— especially those who binge drink. Effects of smaller quantities of alcohol are less clear. Miscarriage rates are i in moderate drinkers. Current advice is to avoid alcohol in pregnancy especially for the first 3mo. If a woman continues to drink, advise her to limit consumption to 1–2u/d. Illicit drugs Cannabis (used by 5% mothers) is possibly associated with poorer motor skills in children and strongly linked with cigarette smoking—discourage. If taking other illicit drugs, refer for specialistcare. Diet  See Box 23.1. Folate supplementation  d risk of neural tube defect (open spina bifida, anencephaly, encephalocele) by72%. For most women, recommend 0.4mg daily from when pregnancy is being planned until 13wk gestation. Recommend 5mg daily if: • Previous child had neural tube defect • Maternal/paternal history or family history of neural tube defect • The mother has coeliac disease, DM, BMI >30kg/m2, or is taking anticonvulsants Only 71 in 3 women take folic acid prior to conception. Effect of starting in early pregnancy is unevaluated. Supplements can be prescribed, are available via the Healthy Start Programme (together with vitamin C and D supplements) or are available OTC from chemists/supermarkets. Introduction of folic acid fortified flour has recently been approved.

Other supplements • Vitamin D The DH recommends 10 microgram (400iu)/d but limited evidence for general use. Consider for women with poor diet or limited exposure to sunlight, those of South Asian, African, Caribbean, or Middle Eastern family origin, and those with pre-pregnancy BMI ≥30kg/m2 • Iron Do not routinely offer—for most, side effects outweigh benefits (b p.822)

Pre-conception and early pregnancy counselling

Box 23.1  Healthy eating tips for women Eat a variety of foods including: • Plenty of fruit and vegetables—at least five portions perday • Plenty of starchy foods, e.g. bread, pasta, rice, or potatoes • Protein-rich foods, e.g. lean meat, chicken, fish, eggs, beans, lentils • Fibre, e.g. wholegrain bread, pasta or rice, fruit, and vegetables • Dairy foods containing calcium, e.g. milk, cheese, and yoghurt Folic acid (folate) Reduces risk of conditions, such as spina bifida. Take folic acid supplements (400 microgram) every day from stopping contraception until you are 13wk pregnant. Eat foods containing folate, e.g. green vegetables, brown rice, fortified bread, and cereals. Iron  Eat iron-rich foods, e.g. red meat, beans, lentils, green vegetables, and fortified cereals. Fruit, fruit juice, and vegetables help with iron absorption.

Food toavoid Pâté and some unpasteurized dairy products All pâtés (including vegetable), Camembert, Brie, other ripened soft cheeses, and blue cheese—may contain Listeria which causes miscarriage, stillbirth, and infections in newborn babies. Raw/undercooked meat, eggs, and ready meals  Risk of food poisoning. • Wash your hands after handling rawmeat • Keep raw meat separate from foods ready toeat • Only eat well-cooked meat—hot right through, with no pink bitsleft • Only eat eggs cooked until white and yolk are solid. Shop mayonnaise and mousses are safe but avoid home-made dishes containing rawegg • Ensure ready meals are piping hot all the way through Liver products and vitamin Asupplements Too much vitamin Acan harm a baby’s development. Avoid eating liver (and liver products, e.g. pâté) and supplements containing vitamin Aor fish liveroils. Some types of fish  Eat ≥2 portions of fish per week (including one of oily fish—mackerel, sardines, fresh (not canned) tuna, or trout)but: • Avoid shark, swordfish, or marlin, and limit tuna to two steaks or four cans weekly. Mercury in these fish can harm a baby’s nervous system • Only eat 1–2 portions of oily fish perweek • Avoid raw shellfish, as they can cause food poisoning Alcohol and caffeine Avoid alcohol. High caffeine levels can cause miscarriage or low birth weight. There is caffeine in coffee, tea, chocolate, cola, and some ‘high-energy’ drinks. You can drink four cups of coffee, six cups of tea, or eight cans of coladaily. Gardening and changing cat litter Toxoplasmosis can harm an unborn baby’s nervous system and/or cause blindness. The parasite that causes it is found in meat, cat faeces, and soil. Wear gloves when gardening or changing cat litter and wash your hands afterwards.

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Sexual intercourse and contraception  Sexual intercourse is not known to be harmful during pregnancy. Often women contemplating pregnancy are still using contraception. Discussion about how to stop/ what to expect may be helpful (e.g. injectables,IUCD). Exercise  bp.792 Chronic disease Review of pre-existing medical conditions with referral for expert a pre-conceptual advice where necessary. • Diabetes mellitus—refer for specialist diabetic review and change women taking sulfonylureas to metformin or insulin (b p.828) • Epilepsy—refer for specialist review of medication (b p.829) • Heart disease—refer for specialist advice if situation is notclear • Genito-urinary disease (e.g. HIV, genital warts, bacterial vaginosis)— treat or refer for treatment/advice on mode of delivery if necessary (b p.810) Review of medication  Drug handling by the body is altered during pregnancy, and drugs can cause damage to the developingfetus: • Discontinue known teratogens prior to conception • Advise to avoid OTC medication unless safety checked with doctor/ midwife • Avoid prescribed medication as much as possible—few medicines have proven safety in pregnancy. If prescribing use well-known and tested drugs at the smallest possible doses, and only when benefit>risk Problems in previous pregnancies • Recurrent miscarriage and/or cervical incompetence (b p.815) • Congenital abnormalities/inherited disorders—pre-pregnancy counselling and detailed advice on genetic screening for high-risk pregnancies is available via regional genetics services Rubella status Rubella in early pregnancy carries a high chance (40–70%) of deafness, blindness, cardiac abnormalities, or multiple fetal abnormalities (b p.806). If rubella status is unknown, suggest it is checked. If not immune, suggest MMR immunization; avoid pregnancy for 3mo afterwards (live vaccine) and recheck immunity after3mo. Flu vaccination Pregnancy i risk of complications and death from seasonal influenza. Offer annual flu vaccination to all pregnantwomen. Work/benefits  Discussion of benefits available during pregnancy (see Table23.1) and employment law (b p.792) is necessary so that women may avoid possible hazards at work, attend for antenatal care, and plan their maternity leave from early in pregnancy.

Discussion of antenatal care and screening available • Brief discussion of antenatal screening (b p.796) and antenatal care procedures (b p.786) allows women to investigate their choices in pregnancy at their leisure • Brief discussion about miscarriage and possibility of infertility allows women to be more confident about asking for help if problems with conception/early pregnancyoccur

Pre-conception and early pregnancy counselling

Table23.1  Benefits available to pregnantwomen Benefit

Eligibility

Statutory Maternity Pay (SMP)

• Worked for the same • Inform employer for 26wk employer at into the 15th wk least 28d before before the babyisdue startingleave • Pregnant at (or have • Mat B1 form had the baby by) the from midwife/ 11th wk before the GP babyisdue • Earning ≥ NI lower earnings limit in the relevant period

How to apply

Benefits gained Paid for up to 39wk (Maternity Pay Period—MPP)—can start any time from 11th wk before the baby is due until the week ofbirth. • 1st 6wk—90% average earnings • 6–26wk—90% of usual earnings or £135.45/wk— whichever is lower

Maternity Allowance (MA)

• Employed/ self-employed for ≥26wk in the 66wk preceding the baby’s due date (test period) • Average weekly earnings of ≥£30/wk for at least 13wk of the testperiod • Do not qualify for SMP (e.g. changed jobs, become unemployed, self-employed)

Apply >26/40 and within 3mo of date MA due to start.Need: • Form MA1 (from Jobcentre Plus offices, employer or M www.gov.uk • MATB1; and, if employed, • Form SMP1 from employer

Paid for 39wk (Maternity Allowance Period—MAP)—can start any time from 11th week before the baby until the day after birth 90% of usual earnings or £135.45/wk— whichever is lower

Sure Start Maternity Grant

• From 11wk before baby is due to 24wk gestation (or live births 80% from 4–6wk—~½ vomit. Occurs at any time of day (‘morning’ sickness in 2–5kg weight loss (hyperemesis gravidarum—1%, pregnancies) admit for rehydration. Peripheral paraesthesia Abnormalities of sensation (e.g. tingling, pins and needles) of hands/feet are common. Reassure. Symptoms usually resolve after delivery. Carpal tunnel syndrome—bp.486. Skin changes Pigmentation (e.g. linea nigra); spider naevi; abdominal striae; chloasma/merasma; palmar erythema. Skin rashes b p.805. Sweating and feeling hot Common. Check apyrexial. If apyrexial, reassure normal in pregnancy. If pyrexial, look for a source of infection. Swelling Fluid retention affects 80%—ankles, hands/fingers, face. If severe/sudden i in oedema, exclude pre-eclampsia (check BP, dipstick urine for protein). Symphysis pubis dysfunction 3%. Symphysis separates causing discomfort/pain in lower abdomen/pelvic area radiating to lower back, upper thighs, and perineum. Pain is constant and worse on movement and resolves on rest. Treat with simple analgesia. Consider referral to physiotherapy for pelvic support belt or elbow crutches. Advise rest in a semi-recumbent position when in pain. Generally resolves after delivery but if persists refer to orthopaedics. Urinary frequency Check MSU—UTI is common in pregnancy and associated with premature delivery—b p.812. vagin*l discharge Usually i in pregnancy. Investigate if smelly, itchy, sore, or associated with dysuria. Varicose veins  Cause aching legs, fatigue, itch, and ankle/foot swelling. If ankles are swollen, exclude pre-eclampsia (check BP, dipstick urine for proteinuria). Elevate legs when sitting, provide support stockings, and encourage walking/discourage standing still. Complications include thrombophlebitis—treat with ice packs, elevation, support stockings, and analgesia—and DVT (b p.824).

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Pruritus and rashes in pregnancy • At booking

• Enquire if the woman has had chickenpox and/or shingles in the past. If not, advise her to make urgent contact if she develops a chickenpox-type rash or has contact with chickenpox or shingles • Advise the woman to inform the midwife/GP urgently if she develops any rash during pregnancy or has contact with anyone who has arash

Contact with rashes in pregnancy Many pregnant women have young children and contact with children with rashes is common. Management—see Figure23.4. Pregnant woman in contact* with rash

Rash is non-specific and non-vesicular, in keeping with possible rubella, measles, or parvovirus B19

Previous history of: measles-containing vaccine x2 or known to be immune Yes

No

Reassure measles unlikely

No Contact has confirmed or likely measles

Detected

Yes Check measles IgG

Rash is vesicular, in keeping with varicella or herpes zoster

No/uncertain past history of varicella or herpes zoster Check varicella IgG

Detected Reassure Not detected

Give VZ-Ig 35wk gestation. Treat with calamine and/or topical steroids. Clears spontaneously 20wk gestation, infection does not affect thebaby. Transmission risk to thebaby • 16wk gestation minimal risk of deafnessonly Transmission risk is much lower with reinfection (10wk, treatment with low molecular weight heparin i live birthrate • Cervical incompetence Diagnosis is usually made on the basis of history of suggestive symptoms in past pregnancies—≥1 late second trimester or early third trimester miscarriage (usually painless leaking of liquor or gradual painless dilatation of the cervix). Refer all women with past history for early obstetric review. Treatment is with cervical USS monitoring with cerclage if cervical length is 35y • IUD(14%) • Smoking • Infertility(15%) History • Abdominal pain (97%). Unilateral or bilateral, may start before bleeding; radiates to shoulder tip; i on passing urine/opening bowels • Amenorrhoea (75%). Peak incidence after 7wk amenorrhoea • Irregular vagin*l bleeding (79%). Described as ‘prune juice’ but may be fresh blood; usually not heavy. May pass decidualcast Examination  Shock in 15–20%; abdominal tenderness 9 rebound or guarding (71%); pelvis—enlarged uterus, adnexal mass, and/or cervical excitation. Management  Admit immediately for further investigation. Resuscitate before admission as needed. Hospital management may be expectant (watch and pregnancy resolves spontaneously), medical (methotrexate), or surgical (laparotomy or laparoscopic surgery). Offer early USS in future pregnancies to confirm pregnancy is intrauterine. Complications  Death if undetected, infertility (pregnancy rate post-ectopic pregnancy is 66% with 10% having a further ectopic pregnancy).

Trophoblastic diseaseG Hydatidiform mole Trophoblastic tumour containing 46 chromosomes (usually of paternal origin) and no fetal material. 8–20% become invasive and penetrate the uterus and/or metastasize to the lungs. Presentswith: • Bleeding in early pregnancy 9 exaggerated symptoms of pregnancy • Uterus is usually large for dates, and no fetal heart can beheard

Bleeding in early pregnancy

• USS has a typical appearance; blood—ii serumβ-HCG • Rarely symptoms of metastatic spread—haemoptysis, pleurisy Refer urgently to gynaecology. If mole is confirmed, women are followed up by specialist centres. Invasive disease requires chemotherapy. Combined hormonal contraception is contraindicated until normal HCG values are obtained. Pregnancy is not advised until completion of the surveillance period—investigate with early USS and β-HCG as incidence of further molar pregnancy is ~1in80. Partial mole Tumour of trophoblast containing 69 chromosomes, 1 maternal and 2 paternal sets, with some fetal tissue. Afetal heart may be seen on early USS but is absent by 8–9wk. Treat as for mole. Rarely becomes malignant (0.5%). Choriocarcinoma  Malignant trophoblastic tumour following molar (rarely normal) pregnancy. Presents with vagin*l bleeding and/or metastases (shadows on CXR, dyspnoea, haemoptysis). Excellent prognosis after treatment with chemotherapy. No contraceptive restrictions after completion of therapy; pregnancy is possible >1y after treatment. Placental site trophoblastic tumour (PSTT)  Rare. Follows 3–4y after normal pregnancy. Presents with abnormal bleeding or amenorrhoea. Can present with distant metastases similarly to choriocarcinoma. Treatment is with hysterectomy 9 chemotherapy. Prognosis isgood. Psychological effects of early loss of pregnancy Broach the subject with all women who have suffered early loss of pregnancy. Include the woman’s partner if possible. • Legitimize grief and acknowledge it—not all women grieve—adjust your approach accordingly; discuss worries/concerns • Provide information about the condition which caused the loss; risk to future pregnancies (if 24wk gestation (or the point of fetal viability) is an antepartum haemorrhage(APH). Bleeding 2cm from the internalos. 0 Women who have anterior placenta praevia on routine anomaly scanning and who have had a previous Caesarean section may have placenta accreta. Maternal complications • APH—typically painless bleeding >20wk with a peak incidence at34wk • Malpresentation—35% breech presentation or transverselie • Placental problems—placenta accreta and percreta especially with a history of previous Caesarean section; abruption • Postpartum haemorrhage Fetal complications  IUGR (15%); premature delivery;death.

Ante- and postpartum haemorrhage

Primary postpartum haemorrhage (PPH) Loss of >500mL blood within 24h of delivery. Affects 1 in 100 deliveries. May occur in the community after home delivery, delivery in a community obstetric unit, or after rapid discharge from a consultant-ledunit. Causes The fourTs: • Tone—uterine atony(90%) • Trauma—of the genitaltract • Tissue—retained products of • Thrombin—clotting disorders conception Risk factors  • Low placenta • Age>40y • Abruption—known or suspected • Obesity (BMI >35kg/m2) • Asian ethnicity • Prolonged labour(>12h) • Anaemia (Hb 4kg) • Retained placenta (b p.819) •Action

• Call emergency ambulance for immediate transfer to hospital • Gain IV access, take blood for FBC and cross-matching, and start IV infusion if possible • Give ergometrine 0.5mg slowlyIV if available • Give high flow O2 via face mask as soon as possible • If the placenta has not been delivered attempt to deliver it by controlled cord traction • Check for trauma and apply pressure to any visible bleeding point/ repair any visible bleeding point. Bimanual pressure on the uterus (rubbing up the fundus) may decrease immediateloss • Some community units keep carboprost 250 micrograms for emergency use. Use if available—1mL by deep IM injection—repeat after 15min 0 Contraindicated in women with asthma

Secondary PPH Excessive blood loss PV >24h after delivery. Peak

incidence: 5–12d after delivery. Cause Postpartum infection—sometimes associated with retained placental tissue orclot. •Action • If the woman is unwell (shocked or toxic) admit to an obstetric unit for further investigation, intravenous antibiotics 9 evacuation of retained products of conception • If bleeding is slight, manage conservatively. Take a vagin*l swab and start oral antibiotics—amoxicillin 500mg tds and metronidazole 400mg tds. Consider referral for USS and/or obstetric review if not settling

Further information RCOG M www.rcog.org.uk • Management of antepartum haemorrhage(2011) • Management of postpartum haemorrhage(2009) • Placenta praevia and placenta praevia accrete and vasa praevia: diagnosis and management(2011)

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Haemolytic disease and rhesus isoimmunization 15% of women are RhD–ve. Development of anti-D antibodies results from fetomaternal haemorrhage (FMH) in RhD–ve women carrying a RhD +ve fetus. In later pregnancies, these antibodies cross the placenta causing rhesus haemolytic disease of the fetus which gets successively worse with each pregnancy. All RhD–ve mothers are tested for D antibodies at booking, at 28wk, and 2-weekly thereafter. Testing is not performed once women are given anti-D prophylaxis. Anti-D titres 3 risk factors): • Age>35y • Long-distancetravel 2 • Obesity (BMI >30kg/m ) • Prolonged labour(>24h) • Parity ≥3 • PPH >1L or blood transfusion • Smoker • Mid-cavity rotational operative delivery • Gross varicoseveins • Current systemic infection • Elective Caesarian section • Pre-eclampsia • Any surgical procedure in the puerperium • Immobility (e.g. paraplegia, symphysis pubis dysfunction)

Further information RCOG M www.rcog.org.uk • Reducing the risk of thrombosis and embolism during pregnancy and the puerperium(2009) • Thromboembolic disease in pregnancy and the puerperium(2007) NICE Antenatal and postnatal mental health (2007) M www.nice.org.uk

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Hypertension in pregnancy Chronic hypertension  Hypertension present 20wk gestation

Action Diastolic BP ≥90 and 262

84

>275

90

>287

96+

>300

Action

Refer for investigation of underlying cause and consideration of phototherapy

0 Treatment thresholds differ according to gestational age at birth. Graphs for all gestational ages are available to download from: M http://guidance.nice.org.uk/CG98/treatmentthresholdgraph/xls/English

Neonatal jaundice

Jaundice persisting >2wk (>3wk in preterm babies) Although 10% of breastfed babies are still jaundiced at 1mo of age, it is important to refer to paediatrics to exclude pathological causes, including: • Hypothyroidism (b p.850) • Haemolysis • Infection—particularly UTI (b p.878) • Liver disease—neonatal hepatitis or biliary atresia Jaundice at any time  If the baby is unwell, has persistently pale, chalky stools and/or dark yellow urine which stains the nappy (baby urine should be almost colourless), then refer. If conjugated bilirubin is >25micromol/L expert advice is essential.

Neonatal hepatitis  Presents with persistent neonatal jaundice. Always requires specialist investigation and management. Possible causes: • Cystic fibrosis • Congenital infection, e.g.HBV • Glycogen storage diseases • Galactosaemia • α1-antitrypsin deficiency

Galactosaemia  Inborn error of metabolism characterized by i plasma galactose. Clinical manifestations depend on enzyme defect: • Galactokinase deficiency Autosomal recessive inheritance. Incidence: 1/40,000. Presents in childhood with cataracts. Treatment involves a galactose-freediet • Classic galactosaemia Autosomal recessive inheritance. Incidence: 1/44,000. The child appears normal at birth but becomes anorexic and jaundiced within a few days or weeks of consuming breast milk or lactose-containing formula. Vomiting, poor growth, hepatomegaly, and septicaemia are common and can be rapidly fatal. Treatment involves eliminating all sources of galactose in the diet. Long-term complications—poor growth, learning difficulty, infertility, speech and neurological abnormalities—are common Biliary atresia  Incidence: 1/15,000. End-stage of a sclerosing process in

an initially patent biliary tree. Cause is unclear. Presents with jaundice in neonates. Prognosis has improved with laparotomy and porto-enterostomy which relieves the problem in 750–70% of babies. 0 Early diagnosis is particularly important so that surgery can be carried out 6mo of age. Use a cup rather than bottle>6mo. Follow-on formula  Contains more iron and casein. Not essential unless a child is not taking solids and is >6mo old. Baby milk suitable from birth can be used until a switch is made to normal cow’smilk. Unmodified cow’s milk  Not recommended until the baby is >1y old as less digestible and contains littleiron. Hydrolysed protein and amino acid infant formulae (e.g. Nutramigen®, Neocate®, Aptamil® pepti) are available on prescription for children with proven cow’s milk protein allergy, lactose intolerance, or galactosaemia. Soya protein-based formula  Not recommended for babies 38.5°C • Cough • Chest and/or abdominalpain • Tachypnoea, recession or other signs of respiratory difficulty • Crepitations, d breath sounds ± bronchial breathing Management • Severe If oxygen saturation 70 breaths/ min aged 50 breaths/min aged >1y), or difficulty breathing/ grunting; dehydrated (or not feeding if 50% boys) or pathological (80% girls). In all cases, refer to a paediatrician for further assessment. Pathological causes: • Failure of the ovaries/testes (p or hypergonadotrophic hypogonadism) • Failure of stimulation of normal gonads to produce sex hormones (s or hypogonadotrophic hypogonadism) Precocious puberty  Puberty before the normal age for the population (in the UK:4 joints. Joints tend to be stiff rather than hot and swollen

Still’s disease* (Systemic onset JIA)

10% of JIA—only 1 in 3 have arthritis at the onset of the disease Affects boys and girls equally up to 5y—then girls are more commonly affected Presentation: •  Fever—high, swinging, early evening temperature for>2wk • Rash—pink maculopapular rash withfever •  Musculoskeletal pain—arthralgia, arthritis, myalgia •  Generalized lymphadenopathy • Hepatosplenomegaly • Pericarditis ± pleurisy (uncommon) Investigations: blood—i ESR/CRP; FBC—i neutrophils, i platelets. Autoantibodies are–ve • Differential diagnosis: malignancy—particularly leukaemia or neuroblastoma; infection

Polyarticular onset JIA

Starts with or without a preceding systemic illness at any age >1y. Usually occurs in teenagers producing widespread joint destruction There is symmetrical arthritis of hands, wrists, PIP joints ± DIP joints Rheumatoid factor is usually–ve (+ve in 3%—often teenage girls)

Enthesisrelated JIA

Affects teenage and younger boys, often producing an asymmetrical arthritis of lower limb joints and tendon insertions. Heel pain and soft tissue swelling are common Associated with HLA-B27 and acute anterior uveitis Represents the childhood equivalent of adult ankylosing spondylitis. 760% of childhood sufferers develop ankylosing spondylitis later in life

Juvenile psoriatic arthritis

Polyarthritis affecting large and small joints, including fingers and toes. The arthritis can be very erosive Psoriasis may be present in the child or a first-degree relative (b p.618)

G.F. Still (1868–1941)—English paediatrician.

*

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Paediatric dermatology Birthmarks Strawberry naevus (capillary haemangioma) • Not usually present atbirth • Occurs anywhere on the skin surface • Starts as a small, red patch, then grows rapidly over a few months into a bright red vascularlump • After initial growth, the naevus stays the same size for 6–12mo, then involutes and disappears by5–7y • No treatment is needed. Parents may need considerable reassurance • If interfering with feeding, breathing, or vision—refer for treatment with intralesional steroids orlaser Port wine stain (naevus flammeus) • Present atbirth • Irregular red/purple macule which often affects one side of theface • Permanent—may become darker and lumpy in middleage • May be associated with other abnormalities, e.g. intracranial vascular malformation (Sturge–Weber syndrome—b p.899) Salmon patch (storkmark) • The most common vascular naevus (750% neonates) • Small, telangiectatic lesion forming a pink macule—most commonly at the nape of the neck or on the upperface • Facial lesions resolve spontaneously—those on the neck may persist. No treatment is needed Mongolian bluespot • Bluish discoloration of the skin, usually over buttocks and lower back in dark-skinned babies • Of no clinical significance but may occasionally be mistaken for bruising and non-accidental injury • Usually disappearsby1y

Congenital melanocytic naevi  71.5% of neonates. • Noted at birth as raised nodules or plaques of black orbrown • May be hairy, irregular, and single or multiple • Classified by size:20cm—large • There is a risk of malignant change to melanoma—the larger the naevus the greater therisk • Laser therapy can improve cosmetic appearance Nappy rash Most common type of nappy eruption. Usually seen in young infants—rare >12mo. An irritant dermatitis due to skin contact with urine or faeces. Presentation  Glazed erythema in the napkin area, sparing skinfolds. Secondary bacterial or fungal infection is common. Differential diagnosis • Seborrhoeiceczema

• Candidiasis

•  Napkin psoriasis

Paediatric dermatology

Management • Advise parents to keep the nappy areadry • Give baby as much time as possible with the nappyoff • Apply moisturiser as soap substitute • Apply a barrier cream between nappy changes—although this may interfere with the action of some modern nappies • Topical treatment with an antifungal combined with hydrocortisone is effective if the nappy rash is not clearing

Infantile seborrhoeiceczema • Starts in the first few weeks oflife • Affects body folds—axilla, groins, behind ears, neck ± face and scalp (‘cradle cap’). Flexural lesions present as moist, shiny, well-demarcated scaly erythema. On the scalp, neck, and behind the ears, a yellowish crust isusual • Treat flexural lesions with emollients and 1% hydrocortisone ointment, or with clotrimazole and hydrocortisonecream • Scalp lesions respond to OTC cradle cap creams

Candidiasis  Often complicates nappy rash or infantile seborrhoeic eczema. Erythema, scaling, and pustules involve the flexures. There may be associated satellite lesions. Treatment is with a topical antifungal, e.g. clotrimazole. Juvenile plantar dermatosis  Presents with red, dry, fissured and glazed skin principally over the forefeet. Sometimes involves the whole sole. Usually starts in primary school years and resolves spontaneously in mid-teens. Due to wearing socks and/or shoes made from synthetic materials. Emollients help but topical steroids are ineffective. Advise cotton socks and leathershoes. Accessory nipples  Commonly seen on the milk line in both male and female infants. Usually small and inconspicuous. No treatment is required. Staphylococcal scalded skin syndrome Rare but serious skin

infection seen in infants/young children. Caused by staphylococcal infection releasing epidermolytic toxin causing widespread erythema/blistering. The epidermis shears off giving the appearance of scalded skin. Admit as a paediatric emergency for IV antibiotics and supportive treatment. 0 Can also affect immunosuppressed adults.

Acne  bp.616 Psoriasis  bp.618 Atopic eczema  bp.606 Scabies  bp.639 Head lice  bp.638 Urticaria  bp.614 Impetigo  bp.632 Warts  bp.634 Molluscum contagiosum  bp.635 Further information Electronic dermatology atlas M www.dermis.net

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Diagnosis of cancer in children Every year in the UK, 1,550 children are diagnosed with and 260 children die from cancer. The most common type of childhood cancer is acute leukaemia (1 in 3) followed by brain tumour (1 in 4). Risk of developing cancer for an individual child is 71:500. 5y survival is 78%; of those that survive 5y, 10% die as a result of tumour recurrence or treatment effects. Diagnosis of childhood malignancy is a particular challenge in primary care. GPs rarely see children with cancer, and the cancers that children get are often unfamiliar to them. Always have a high index of suspicion and if in doubt refer for a specialist opinion. Referrals should be made to a paediatrician or specialist in children’s cancer. 0 Some congenital/genetic syndromes may be associated with i risk of childhood cancer (e.g. Down’s syndrome—leukaemia; neurofibromatosis and tuberous sclerosis—CNS tumours).

Abdominal distensionN  If persistent or progressive, examine the abdomen: • If a mass is found, refer immediately • If the child is uncooperative and abdominal examination is not possible, or if examination is difficult, consider referral for urgent abdominalUSS

Unexplained or persistent back painN  Examine the child and check FBC and blood film. Consider X-ray and/or discussion with a paediatrician. If no cause is found, refer urgently. Admission or referral to be seen the same dayN  Any childwith: • Hepatosplenomegaly • Unexplained petechiae • Unexplained urinary retention • d consciouslevel • Headache and vomiting that cause early morning waking or occur on waking, as these are classic signs of i intracranial pressure 0 1 type may occur concurrently PHYSICAL Hitting, shaking, throwing, burning, suffocating, poisoning, including fabricated or induced illness

EMOTIONAL The child is made to feel worthless, afraid, unloved, or inadequate. Includes age-inappropriate expectations and witnessing domestic violence

NEGLECT Failure to meet the child’s basic needs (including medical needs); allowing the child to be exposed to danger

SEXUAL Forcing/enticing a child to participate in sexual activities—physical contact or production of p*rnographic material

Circumcision of female children or forced marriage 5. Common in patients who smoke, patients with ill-fitting dentures, and patients who habitually chew on their cheek. Usually benign but may be an early sign of oral cancer. NICE recommends referral to oral surgery to exclude malignancy in all casesN. Erythroplakia Reddened area that results when the lining of the mouth thins. The area appears red because the underlying capillaries are more visible. Erythroplakia is a much more ominous predictor of oral cancer than leukoplakia. NICE recommends referral to oral surgery to exclude malignancy in all casesN. Tongue problems • Blue tongue Central cyanosis—bp.232 • Dry and furred tongue Suggests dehydration • Geographic tongue Irregular smoother redder patches that change position over time on the dorsum of the tongue. Due to papillae loss. Asymptomatic or causes soreness. Rarely due to vitamin B12 deficiency • Large tongue Consider acromegaly, amyloidosis, myxoedema

Themouth

• Smooth tongue Iron, riboflavin, nicotinic acid, B12 or folate deficiency; idiopathic—usually elderly; antibioticuse • Sore tongue Glossitis of anaemia; Crohn’s disease; coeliac disease; carcinoma of the tongue; psychogenic causes • Strawberry tongue Yellowish white tongue coating with the dark red papillae of the tongue projecting through. Associated with scarlet fever although also present in Kawasaki’s disease • Ulcer Assume any non-healing ulcer is due to carcinoma of the tongue until proven otherwise. Refer for biopsy to an oral surgeon. Treatment is with surgery or laser ablation ± radiotherapy Halitosis Common after sleep. Short-term halitosis is associated with acute illness, e.g. tonsillitis, appendicitis (foetor oris), gastroenteritis, diabetic ketoacidosis.

Chronic halitosis  Is usually caused by bacterial putrefaction of food debris and dental plaque and is related to poor oral hygiene. Associated with gingivitis 9 periodontitis. Smoking, alcohol, isosorbide dinitrate, and disulfiram exacerbate the problem. Rarely caused by metabolic disorders, e.g. trimethylaminuria (TMAU or fish odour syndrome). Management  Examine the mouth and recommend a dental check. Advise oral hygiene, e.g. regular brushing of teeth/tongue, dental flossing; smoking cessation; diet advice—avoid garlic, onions, curries; treat any local infection, e.g. gingivitis; mouthwashes, e.g. 0.2% aqueous chlorhexidine gluconate help d dental plaque. Refer if causing distress. Aphthous ulcers Painful white ulcers. Common, affecting ~20%. Usually idiopathic but may be associated with poor health, stress, Crohn’s, coeliac, and Behçet’s disease. Most are short-lived. Large ulcers (up to 2cm diameter) can take ~6wk to heal. Most resolve spontaneously. Topical therapies are effective, e.g. hydrocortisone lozenges qds (dissolve in contact with the ulcer). If ulcers are recurrent, check FBC, iron, and folate levels. Refer any ulcer not significantly improving >3wk after presentation to exclude malignancy, or if recurrent ulcers cause distress. Oral cancer >6,200 new cases/y in the UK. Incidence is increasing. Usually squamous cell carcinoma. 4 > 5. Major risk factors are smoking and high alcohol consumption. Lip cancer has good prognosis (>90% 5y survival), but overall survival is poor (51% 2y survival) mainly due to poor public awareness/late presentation. Usually presents with leukoplakia (white patch), erythroplakia (red patch), or non-healing ulcer (>3wk). Management  Refer suspicious lesions to oral surgery for biopsy.

Lichen planus  bp.620 Erythema multiforme  bp.597 Oral thrush  bp.636 Behçet’s disease  bp.519 Herpes simplex virus (HSV) infection (cold sores)  bp.634 Further information NICE Referral guidelines for suspected cancer (2005) M www.nice.org.uk

Support and information for patients Mouth Cancer Foundation F 01924 950 950 M www.mouthcancerfoundation.org

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Dental and jaw problems Gums • Bleeding gums Consider periodontal disease (most common cause); pregnancy; leukaemia, bleeding disorders; scurvy • Hypertrophied gums Associated with phenytoinuse • Blue line Along the margin of the teeth—suggests lead poisoning • Gum inflammation Gingivitis—consider immunodeficiency; vitamin C deficiency; DM, leukaemia; drugs; e.g. phenytoin, nifedipine, ciclosporin Vincent’s angina Pharyngeal infection with ulcerative gingivitis. Management: penicillin V 250mg qds po + metronidazole 400mg tds po. J.H. Vincent (1862–1950)—French bacteriologist. Periodontal disease  Disease of the periodontal ligament caused by bacterial plaque and exacerbated by smoking and DM. Occurs in the normal population >30y. Leads to gingivitis, dental abscesses, and tooth loss. Encourage patients to register with a dentistN—regular dental care helps prevent dental emergencies and periodontal disease. Patients experiencing difficulty finding an NHS dentist should telephone their local Primary Care Organization and ask to be found a dentist. • Refer urgently to a dentist if unexplained tooth mobility for>3wkN.

Toothache Pain/excessive sensitivity to temperature may be a problem with exposed dentine or pulp infection—advise to see a dentist. Dental abscess  Facial swelling and pain related to bacterial infection. Refer to a dentist. Prescribe analgesia if there will be adelay. Complications of tooth extraction • Haemorrhage Apply pressure by placing wet gauze over socket and get patient to bite hard for 15min—refer to dentist if not stopping • Painful socket and bad taste in mouth Infection—refer to a dentist. Give analgesia if delay is likely Loss of tooth through trauma  b p.1108 Cleft lip and palate  Incidence: 1:600 live births—half have other abnormalities too (e.g. hypoplastic mandible). Often—though not always—detected at routine antenatal USS. The cleft may be unilateral or bilateral and involve lip and/or palate. Cleft lips are usually repaired in the first few days of life; cleft palates at 73mo depending on the weight of thebaby. Problems associated with cleft lip and/orpalate • Feeding difficulties with associated poor weightgain • Aspiration pneumonia • Hearing problems—particularly glue ear. In some areas children with cleft palate are routinely given grommets at 718mo. Treat otitis media promptly. Audiology review is important • Speech problems—refer for speech therapy • Dental problems—universal with cleft palate. Orthodontic treatment is always required

Dental and jaw problems

Temporomandibular joint (TMJ) dysfunction Common disorder affecting 770% of the population—only 5% seek treatment. Typically presents in early adulthood. 4:5 8 1:4. Aetiology is complex—malocclusion and trauma play a part and are exacerbated by psychogenic factors. Assessment  Take a careful history noting pain—duration, location, and nature; precipitating/relieving factors; joint noises; restricted jaw function, e.g. locking, poor bite; and non-specific symptoms, e.g. headache, earache, and tinnitus. Examine the head and neck, including the TMJ and mandibular movement. Exclude other disease. Do not X-ray as this yields little useful information—CT/MRI may be ordered by specialists. Patterns of disease  There are 3 patterns of disease: • Myofacial pain and dysfunction Due to clenching/teeth grinding. Pain is usually worse in the morning. Stress, anxiety, and depression are key features. Poor sleep is common. May have diffuse muscle tenderness • Internal derangement The articular disc is in an abnormal position and causes restriction of mandibular movement. Pain is usually continuous and exacerbated by jaw movement • Osteoarthrosis Degeneration of the joint seen on older patients. Crepitus and sounds from the joint occur on jaw movement Management  Reassure and explain the benign nature of the disorder. Suggest simple analgesia, e.g. paracetamol ± ibuprofen. Resting the jaw and avoiding stress may help. Refer those with ongoing problems to oral surgery. Specialist treatment A bite appliance to wear at night helps 70%. Physiotherapy, behavioural therapy, and exercises also help. Drug treatments include NSAIDs, antidepressants, opioids, and muscle relaxants. Surgery is occasionally necessary if medical treatmentfails.

Dislocated jaw  b p.1113

Fractured mandible  b p.1113

Information and support British Association of Oral and Maxillofacial Surgeons Salivary gland disorders; temporomandibular joint disorders M www.baoms.org.uk Cleft Lip and Palate Association (CLAPA) F 020 7833 4883 M www.clapa.com

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Sorethroat Each GP sees ~120 patients with sore throat every year—mostly children and young adults. 70% sore throats are viral in origin—the rest bacterial (mostly Group AB-haemolytic streptococci).

Clinical picture Pain on swallowing; fever; headache; tonsillar exudates;

nausea and vomiting; and/or abdominal pain (especially in children due to abdominal lymphadenopathy). 0 Viral and bacterial infections are indistinguishable clinically but association with coryza and cough may point to a viral aetiology.

Differential diagnosis  Glandular fever especially in young adults, with

persistent sore throat.

Investigation  Not usually undertaken. • Throat swabs cannot distinguish commensal organisms from clinical infection (40% carry Group AB-haemolytic streptococci), are expensive and do not give instant results so are rarelyused • Rapid antigen tests give immediate results but have low sensitivity limiting usefulness

Management  90% recover in 1moN. Referral for tonsillectomyG • Recurrent acute tonsillitis Young children have a lot of throat infections, and most will ‘grow out’ of the problem without the need for surgery. Tonsillectomy is only considered if children miss a lot of school, e.g. >5 attacks/y for 2y causing school absence • Airway obstruction Very large tonsils causing sleepapnoea • Chronic tonsillitis >3mo + halitosis • Recurrentquinsy • Unilateral tonsillar enlargement To exclude malignancy. • Tonsillectomy carries a small risk of severe haemorrhage. Readmit any patient with bleeding post-operatively for observation.

Glandular fever (infectious mononucleosis) Consider in teenagers or young adults presenting with sore throat lasting >1wk. Caused by Epstein– Barr virus (EBV). Spread by droplet infection and direct contact (‘kissing disease’) and has a 4–14d incubation period. Presents with sore throat, malaise, fatigue, lymphadenopathy, enlarged spleen, palatal petechiae, and/ or rash (10–20%). Send blood for FBC (atypical lymphocytes) and glandular fever antibodies (Monospot or Paul Bunnell). Management  Advise rest, fluids, and regular paracetamol, avoid alcohol. Try salt water gargles or aspirin gargles (only if >16y). Consider a short course of prednisolone for severe symptoms. Treat 2o infection with antibiotics. Counsel re the possibility of prolonged symptoms (up to several months). • Do not prescribe amoxicillin as it causes arash. Complications  2o infections; rash with amoxicillin; hepatitis; jaundice; pneumonitis; neurological disturbances (rare). Tonsillar tumour  Most often elderly. Signs: unilateral tonsillar swelling, dysphagia, sore throat, earache. Refer for excision biopsy. • Refer any unexplained, sore throat present for >1mo for urgent ENT assessmentN.

Further information NICE M www.nice.org.uk • Respiratory tract infections:antibiotic prescribing(2008) • Referral guidelines for suspected cancer(2005)

Information for patients Patient UK Information leaflets on sore throat. URTI, tonsillitis, tonsils and adenoids, and glandular fever M www.patient.co.uk

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Hoarseness and stridor Hoarseness Change in quality of the voice affecting pitch, volume, or resonance. Occurs when vocal cord function is affected by a change in the cords, a neurological or muscular problem. Causes: • Local causes URTI (most common); laryngitis; trauma (shouting, coughing, vomiting, instrumentation); carcinoma; hypothyroidism; acromegaly • Neurological problems Laryngeal nerve palsy; motor neurone disease; myasthenia gravis; multiple sclerosis • Muscular problems Muscular dystrophy • Functional problems Assessment  Weight d, dysphagia, or neck lumps add to suspicions of malignancy. Check TFTs in those with weight gain. Indirect laryngoscopy with a mirror can be difficult and give a poor view. ENT departments have thin fibreoptic scopes for direct visualization in outpatients. • Refer urgently for chest X-ray (CXR)N If hoarseness persisting >3wk—particularly smokers >50y and heavy drinkers. If there is a POSITIVE finding on CXR Refer urgently to a team specializing in the management of lung cancer. If there is a NEGATIVE finding on CXR Refer urgently to a team ­specializing in the management of head and neck cancer.

Laryngitis Hoarseness, malaise 9 fever and/or pain on using voice.

Usually viral and self-limiting (1–2wk) but occasionally 2o bacterial infection occurs. Management  Advise patients to rest voice, take OTC analgesia, e.g. paracetamol and/or ibuprofen, try steam inhalations. Consider antibiotics if bacterial infection is suspected (e.g. phenoxymethylpenicillin 250mg qds for1wk).

Vocal cord nodules Can cause hoarseness. Usually precipitated by overuse of the voice—typically in singers. They can be visualized at laryngoscopy. Initial treatment is resting the voice but sometimes nodules have to be removed surgically. Functional disorders  Hysterical paralysis of the vocal cord adductors due to psychological stress. Can cause the voice to d to a whisper or be lost completely. More common amongst youngwomen. Management  Refer for laryngoscopy to exclude organic cause. Speech therapy and psychological support mayhelp.

Laryngeal carcinoma  4 > 5. Smoking is the main risk factor. The first sign is usually hoarseness, followed by stridor, dysphagia, andpain, Management  Refer urgently to ENT if suspected. Diagnosis is confirmed with laryngoscopy and biopsy. Treatment is with surgery 9 radiotherapy. Early tumours confined to the vocal cord have 80–90% 5y survival.

Hoarseness and stridor

Post-laryngectomy problems After laryngectomy patients have a permanent tracheostomy and require practical and psychological support. Problems include: • Excessive secretions    • Recurrent pneumonia • Stenosis of the tracheostomy site—refer to ENT/oral surgery if severe • Communication difficulties—ensure referred to speech therapy • Maintenance of adequate diet—refer to dietician if not maintaining weight and recurrence of tumour has been excluded Stridor  Noise created on inspiration due to narrowing of the larynx or trachea—much more common in children than adults. Treat thecause. • Signs of severe airway narrowing • Use of accessory muscles and • Distress trachealtug • i respiratoryrate • Pallor and cyanosis Causes  Congenital abnormalities of the larynx; epiglottis; croup (laryngotracheobronchitis); inhaled foreign body; trauma; laryngeal paralysis.

Laryngomalacia (congenital laryngeal stridor) Common among small babies. Due to floppy aryatic folds and the small size of the airway. Stridor becomes more noticeable during sleep, excitement, crying, and with concurrent URTIs. Normally resolves without treatment. Parental concern may necessitate referral. Croup Common viral infection occurring in epidemics in autumn and spring. Starts with mild fever and runny nose. In younger children (2cm insize • Lymphadenopathy • Widespread lymphadenopathy present≥6wk • LNs are increasing insize • Associated weight d, night sweats, and/or splenomegaly Causes of lymphadenopathy • Benign infective Viral infection, e.g. EBV, CMV, adenovirus, HIV; bacterial infection, e.g. streptococcal sore throat, TB; toxoplasmosis; syphilis • Benign non-infective Sarcoid; connective tissue disease (e.g. RA); skin disease (e.g. eczema, psoriasis); drugs (e.g. phenytoin) • Malignant Lymphoma, CLL, ALL, metastases—head and neck cancer may present with enlarged cervicalLNs Branchial cyst Arises from embryonic remnants of the second branchial cleft in the neck. Most common in young adults. Presents as a smooth swelling in front of the anterior border of the sternomastoid at the junction of its upper and middle thirds—often during a viral URTI. Position is characteristic. Examination: fluctuant lump that does not move on swallowing. Treatment is by excision—refer toENT. Thyroglossal cyst  The thyroid gland develops from the lower portion of the thyroglossal duct. If a portion of this duct remains patent it can form a thyroglossal cyst. Usually presents in young adults (peak age 15–30y) with either a painless, smooth, cystic midline swelling between the isthmus of the thyroid gland and the hyoid cartilage or just above the hyoid cartilage or, if the cyst is inflamed, a painful, tender lump with localized swelling. Examination: the cyst rises as the patient sticks out his tongue. Refer to ENT for excision.

Salivary gland strictures andstones • Salivary stones 80% of calculi are seen in the submandibular duct system. Less frequently they occur in the parotid duct system and rarely in other salivary glands • Strictures of the salivary gland duct occur as a complication of a pre-existing calculus, due to mucus plugs or following trauma to the duct wall (e.g. cheek biting)

Neck lumps and salivary gland problems

Lump in the neck

Consider: • Sebaceous cyst • Lipoma • Carbuncle • Neurofibroma

YES

Is the lump superficial to the underlying muscle and fascia? NO Is the lump in the midline?

YES

NO Consider: • Lymph nodes • Branchial cyst • Cystic hygroma • AV fistula • Thyroid swellings (b p. 362) • Carotid artery aneurysm (b p. 285) • Spinal abscess • Cervical rib (b p. 475) • Carotid body, sternomastoid, or salivary gland tumour

Consider: • Dermoid cyst • Thyroglossal cyst (moves on sticking out tongue) • Pharyngeal pouch (b p. 385) • Laryngocele • Subhyoid bursa • Plunging ranula • Carcinoma of the larynx, trachea, or oesophagus

Figure25.1  Differential diagnosis of necklumps Presentation  Pain and swelling on eating due to obstruction of saliva flow. The gland may appear normal or be tender and swollen. Sometimes stones can be visualized at the salivary duct orifice or felt on bimanual palpation. Both stones and strictures predispose to infection in thegland. Management  Refer to ENT/oral surgery for confirmation of diagnosis— stones are seen on plain X-rays or sialography. Some stones pass spontaneously but most require surgical removal—the whole gland may be removed to prevent recurrent problems. Strictures can often be dilated. Acute parotiditis Unilateral parotid swelling and pain caused by bacterial infection. Predisposing factors include DM, immunosuppression/ compromise, local fibrosis following radiotherapy, and autoimmune destruction (e.g. Sjögren’s). Precipitating factors include surgery, dehydration, salivary stones/strictures, and poor oral hygiene. Treat with antibiotics (e.g. amoxicillin 500mg tds for 1wk) and rehydration. If not settling consider abscess formation—refer to ENT/oral surgery for drainage.

Mumps  bp.652 Salivary gland tumours Present with a lump/swelling in a salivary

gland—80% in the parotid gland. Treated with surgery ± radiotherapy.

• Refer urgently to ENT/oral surgery if unexplained swelling in the parotid or submandibular gland for >1moN. Refer sooner if pain, rapid growth, hard fixed mass, weight d, or facial nervepalsy.

Further information NICE Referral guidelines for suspected cancer (2005) M www.nice.org.uk

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Nasal problems Anosmia

Bilateral anosmia  More common than unilateral. Causes: • Local causes URTI, rhinitis, enlarged turbinates, nasal polyps • Central causes CNS tumours, after head injury, meningitis, hydrocephalus, Kallman’s syndrome Unilateral anosmia One-sided loss of the sense of smell. Causes: head injury, frontal lobe lesion. Taste disturbance  Taste of food is often dependent on smell. Any cause of anosmia can also result in taste disturbance. Other causes of taste disturbance include: • Drugs—taste disturbance is a side effect of ACE inhibitors • Glossopharyngeal nerve palsy—taste loss on posterior third of thetongue • Facial nerve palsy (b p.537) • Chronic adrenal insufficiency—i sensitivity totaste • Malignancy—taste sensations, e.g. metallic taste with pancreaticcancer

Nasal discharge • Clear discharge May be physiological (e.g. due to cold air), due to allergy (e.g. hay fever), or viral (e.g.URTI) • Clear fluid after trauma Can indicate CSFleak • Green discharge Indicates active bacterial infection • Yellow discharge May indicate viral/bacterial infection or allergy • Persistent blood stained discharge • Tumour of the nose or post-nasal sinus until proven otherwise—refer urgently toENT 0 The term rhinorrhoea is also used to mean nasal discharge. The term coryza is usually applied to watery discharge from nasal mucus membranes that occurs when a patient has a viral infection. CSF rhinorrhoea Clear fluid dripping from the nose after trauma can indicate a fracture of the roof of the ethmoid labyrinth and CSF leak. Fluid tests +ve for glucose. It suggests significant trauma—consider referral for head injury assessment. Spontaneous healing of the CSF leak is the norm but if it persists refer to neurosurgery for dural closure. Nasal obstruction Common symptom experienced occasionally by many. Usually obstruction is bilateral. • Assume persistent unilateral blockage is neoplastic until proven otherwise—refer urgently toENT. Causes of nasal obstruction • Mucosal swelling Coryza, rhinitis (b p.942), iatrogenic, nasalpolyps • Septal deviation Trauma, congenital, e.g. 2o to cleftlip • Other Tumour, enlarged adenoids, foreign body (b p.1108)

Deviated nasal septum Common in adults—usually s to injury. May

be associated with external deformity. Nasal blockage is unilateral. Treat mucosal swelling due to rhinitis first as that may be sufficient to control symptoms. If unsuccessful, refer for surgery (submucous resection).

Nasal problems

Septal haematoma  May occur after injury and causes nasal blockage.

Presents as a bilateral soft bulging of the septum. Refer urgently to ENT for evacuation to prevent cartilage destruction. Septal perforation Can cause bleeding, crusting, and discomfort. Causes: trauma, nose picking, cocaine use, post-operative, malignancy. Refer if suspicion of malignancy, otherwise treat symptomatically (e.g. vaseline or naseptin for crusting)—surgical closure is often not successful. Post-nasal drip Draining of nasal secretions down the back of the throat. Treat as for chronic sinusitis (b p.942). Symptoms include: • Feeling of mucus in the back of thethroat • Chronic cough—usually worse in the morning and improves in theday • Morning sorethroat • Nasty taste in the mouth/badbreath Causes  URTI, sinusitis, allergic and/or vasomotor rhinitis, nasal polyps, deviated nasal septum. Nasal polyps  Most common in 4 aged >40y—associated with asthma, allergic rhinitis, and chronic sinusitis. Consider CF in children 3mo of symptoms or >3 episodes of sinusitis in any year. Presents with post-nasal drip; frontal headache/facial pain; and/or blocked nose. Associated with nasal polyps (b p.941) and vasomotor rhinitis. Treat as for acute sinusitis. Refer to ENT if symptoms are interfering with life—surgery mayhelp. Rhinitis Inflammation of the nasal mucosa. Affects >1:5 people. May be allergic (most children; 1:3 adults) or non-allergic (e.g. vasomotor—triggered by physical/chemical agents such as cold air, tobacco, or perfumes; drug-induced). If allergic cause is suspected, ask about potential allergens:pollen, animals, fungi/moulds, occupational allergens (e.g. flour, latex). Symptoms  Nasal discharge, itching, sneezing ± nasal blockage/congestion. Symptoms may be seasonal (only certain times of the year) or perennial (all year); intermittent (1mo (e.g. fluticasone, mometasone). If nasal irritation, sore throat, or nosebleeds switch to a preparation without benzalkonium chloride preservative, e.g. Flixonase Nasules® or Rhinocort® Oral steroids Only rarely needed. Consider for: severe nasal obstruction; short-term rescue medication for uncontrolled symptoms; or control of symptoms for important social/work events (e.g. examinations) Use 20–30mg prednisolone po for 5–7d in combination with nasal steroids. Injected preparations are not recommended Oral antihistamines Choose a non-sedative antihistamine, e.g. loratadine 10mg od. May be used alone or in combination with nasal steroids. Improve associated symptoms (e.g. conjunctivitis) as well as nasal symptoms Topical e.g. Azelastine nasal drops—useful as a rescue therapy antihistamines Faster acting than oral antihistamines—onset of action is in > oral preparations) in reducing nasal congestion Discourage use of nasal drops for >10d as vasoconstriction l mucosal damage l worsening of nasal congestion—a vicious cycle termed rhinitis medicamentosa. Not caused by oral preparations Topical e.g. Ipratropium bromide nasal spray tds—d rhinorrhoea but no effect on anticholinergics other nasal symptoms Topical chromones e.g. Sodium cromoglicate or nedocromil sodium nasal spray—less effective than nasal steroids but may be useful for children or pregnant women wishing to avoid steroids

Non-allergic rhinitis  Treat as for allergic rhinitis—treatment is often less successful.

Hay fever Rhinitis and/or conjunctivitis and/or wheeze due to an

allergic reaction to pollen. Occurs at different times in the year depending on which pollen is involved (see Table25.1). Management  When the pollen count is high—keep windows shut (including car windows—consider pollen filter for the car); wear glasses/ sunglasses; avoid grassy spaces. Treat as for allergic rhinitis. Topical chromone eye drops (e.g. nedocromil) may help eye symptoms.

Further information British Society for Allergy and Clinical Immunology (BSACI) Guidelines for the management of allergic and non-allergic rhinitis (2008). See Mwww.bsaci.org

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Earache and external ear problems Earache  Ear pain is a common presenting symptom. Thinkof: • Local causes Outer ear: otitis externa; furunculosis; impacted wax; pinna pain (perichondritis); malignant disease of the ear. Middle ear: otitis media; barotrauma; myringitis; mastoiditis • Referred pain Trigeminal nerve (dental abscess/caries, impacted molar teeth, TMJ dysfunction); facial nerve (HSV infection, Ramsay Hunt syndrome); vagus nerve (tumours of the piriform fossa, larynx, or post-cricoid area); glossopharyngeal nerve (tonsillitis/quinsy/post-op tonsillectomy, tumour of the base of the tongue or tonsil, neuralgia); cervical nerves C2/3 (cervical sponylosis) • Refer urgently to ENT if unilateral unexplained pain in the head/neck area for >4wk, associated with otalgia (earache) but normal otoscopyN.

Myringitis  Inflammation of the tympanic membrane. Myringitis bullosa describes painful vesicles on the tympanic membrane associated with mycoplasma or viral URTIs. Asimilar picture occurs with Ramsay Hunt syndrome (b p.538). Discharge from the ear  Otorrhoea is discharge from the ear. Major causes are:otitis externa; otitis media; and cholesteatoma. 0 Always exclude a perforated drum in discharging ears—beware of cholesteatoma. If you cannot visualize the drum—review the patient. Clear fluid leaking from an ear after head injury may suggest a CSF leak. Fluid tests +ve for glucose. This implies a head injury with force—refer to A&E for further assessment. Otitis externa Inflammation ± infection of the external ear canal. Common—affecting ~10% at some time. Adults > children. Associated with eczema of the ear canal. Risk factors include: swimming, humid environment, narrow ear canal, hearing aid use, and mechanical trauma (e.g. cleaning ears out with cotton buds or after syringing). Acute otitis externa  3mo duration) Ongoing discharge from the ear 9 hearing loss. Causes canal stenosis and permanent hearingd. Management  Although very common, can be difficult to treat. Take a swab if any discharge. • Advise analgesia, e.g. paracetamol 9 ibuprofen • Prescribe ear drops—options are:aluminium acetate drops (as effective as antibiotics); and antibiotic and/or steroid drops (e.g.Locorten-Vioform®) X If you cannot see the eardrum to ensure that it is intact, use of potentially ototoxic gentamicin ear drops is controversial

Earache and external ear problems

• Adding oral antibiotics (e.g. flucloxacillin/erythromycin qds) does not improve outcomeCE—only use if treatment with drops alone has failed or administration of ear drops may be ineffective, e.g. debris within the canal, very swollen canal, uncooperativechild 0 Skin of the pinna adjacent to the ear canal is often affected by eczema. Treat with topical corticosteroid cream/ointment—avoid prolongeduse. If no response after 1wk, Try alternative ear drop, e.g. Otosporin® (contains neomycin, hydrocortisone, and an antifungal—polymyxin B) 9 oral antibiotics. If swab result is available, prescribe based on the result. Consider gentle syringing to remove infected material. Refer to ENT for aural toilet/advice on further management if no response. Furunculosis  Boil in the ear canal. Presents with severe ear pain—may be exacerbated by moving the tragus or opening the jaw. ExcludeDM. • If no surrounding cellulitis—advise OTC analgesia and application of hot compresses; most will settle. If not settling, prescribe topical antibiotics and steroid drops, e.g. Gentisone HC®, 3 drops qds for1wk • If surrounding cellulitis—prescribe flucloxacillin 250–500mg qds for7d • Refer to ENT for incision and drainage if not settling Foreign bodies in the ear  b p.1108 Ear wax  Normal. Becomes a problem only if causes deafness, pain, or other ear-related symptoms. Factors preventing normal extrusion of wax from the ear (e.g. wearing a hearing aid, using cotton buds to clean ears) i the chance of ear wax accumulating. Ear syringing  Indicated if impacted wax causes loss of hearing, discomfort, or tinnitus. Avoid syringing if there is deafness in the other ear or a history of perforation of the eardrum (including grommet), previous mastoid operation, or chronic middle ear disease (e.g. chronic suppurative otitis media, cholesteatoma). If ear syringing is contraindicated, refer to ENT for removal under direct vision, e.g. with microsuction. Perichondritis of the pinna  Infection of the pinna due to ear piercing or laceration. If not treated quickly can result in destruction of cartilage and ‘cauliflower ear’. Pseudomonas is a common infecting organism so treat with oral ciprofloxacin 500–750mg bd. If not settling, refer as an emergency toA&E. Chondrodermatitis nodularis helicis (CNH) Caused by pressure on the ear (e.g. against pillow at night, tight headwear). Tender lump often with overlying scaling/ulceration on the outer helix of the pinna. Tender to lie on and painful in the cold. Advise relief of pressure 9 topical steroid/ antibiotic cream. If this fails, cryotherapy or surgical excision is effective. Differential diagnosis: SCC, BCC, gouty tophus. Haematoma of the pinna  b p.1113

Accessory auricle  Incidence: 1.5:100 live births. Small

skin lesion consisting of skin ± cartilage in front of the ear. No treatment is necessary but accessory auricles are often removed for cosmetic reasons. Bat ears Common congenital abnormality. Afold of the pinna is absent. The child is noted to have protruding ears. Runs in families. Referral for surgery is indicated if the condition is causing psychosocial problems.

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Otitismedia Acute suppurative otitis media (OM) Common, acute inflammation of the middle ear. Parental smoking i children’s risk of OM—encourage parents to stop smoking. Caused by viral/bacterial infection. Presentation Ear pain—usually unilateral ± fever/systemic upset. Ear discharge may be associated with relief of pain if there is a spontaneous perforation of the eardrum. Examination:red, bulging drum. If perforation has occurred the external canal may be filled with pus obscuring the drum. 0 If you can’t see the drum, review the patient after treatment. ManagementN • In 80%, symptoms resolve in ≤4d without treatment. Advise fluids and paracetamol and/or ibuprofen for analgesia and fever control. Symptoms resolve 24h earlier with antibiotics but antibiotics carry the risk of side effects and use i community antibiotic resistance. Consider using a ‘delayed’ approach—prescribing if symptoms are no betterin4d • Consider prescribing immediately (e.g. amoxicillin tds) for children with bilateral OM or acute OM with otorrhoea • Prescribe immediately if very systemically unwell or at high risk of serious complications because of pre-existing co-morbidity, e.g. significant heart, lung, renal, liver, or neuromuscular disease, immunosuppression, CF, young children born prematurely • If recurrent attacks (>4 episodes in 6mo) or if acute perforation does not heal in 1mo) from the ear associated with tympanic membrane perforation and conductive hearing loss. Not usually painful. • Central perforation ‘Safe disease’. Treat as for otitis externa (bp.944). Refer to ENT if there is persistent discharge, deafness, vertigo, or earache. Surgery to close the drum mayhelp • Attic or marginal perforation ‘Unsafe disease’. May indicate cholesteatoma. Refer to ENT for further assessment Serous/secretory otitis media (glue ear) Non-infected fluid accumulates in the middle ear due to dysfunction/obstruction of the Eustachian tube, e.g. s to throat or ear infection, or tonsillar hyperplasia. Most common cause of hearing loss in childhood. More common in children with Down’s syndrome or cleft lip/palate. Symptoms: deafness 9 earache, difficulties with speech/language, ± behavioural problems. Signs: dull, concave drum with visible peripheral vessels ± fluid level and/or air bubbles behind thedrum. Management in children Untreated, 75% have no symptoms

in 12mo. Glue ear resolves as the child grows older—treatment is aimed at d impact of symptoms until natural resolution. If not resolving, refer to community audiology/ENT depending on local policy. Specialist treatment options include watchful waiting or grommet insertion.

Otitismedia

Grommets  Air-conducting tubes inserted through the eardrum to drain the middle ear. Most are extruded spontaneously 1y.

Tinnitus and vertigo

Benign positional vertigo Recurrent attacks of sudden-onset vertigo, lasting only a few seconds or minutes. Occur with sudden changes in posture. Common after head injury or viral illness. Possibly caused by otoliths in the labyrinth. Diagnosis is based on history and a +ve Hallpike test. Normal tympanic membrane. Management  Usually self-limiting (few weeks)—although may continue intermittently for years. Reassure. Labyrinthine sedatives are not helpful. Teach the patient to minimize symptoms by sitting and lying in stages. Habituation may occur by maintaining the trigger position until vertigo settles. If not settling, perform or refer to ENT for Epley’s manoeuvreC (rapid repositioning of head to move otoliths out of the labyrinth) and/or refer to physiotherapy for exercises/vestibular rehabilitation. Viral labyrnthitis  Usually follows a viralURTI. • Symptoms and signs Sudden onset of vertigo, prostration, nausea and vomiting, no associated loss of hearing, normal tympanic membrane • Treatment Labyrinthine sedatives, e.g. cyclizine or prochlorperazine • Natural history Usually resolves in 2–3wk—if persists >6wkrefer Ménière’s syndrome  Overdiagnosed in patients with recurrent vertigo and deafness. It is a complex of symptoms including clustering of attacks of vertigo and nausea, tinnitus, a sense of fullness in the ear, and sensorineural deafness which may be progressive. Aetiology: idiopathic dilation of endolymphatic spaces. Management • Refer all suspected cases to ENT or neurology to confirm diagnosis • Provide information and advise about support organizations • Treat acute attacks with labyrinthine sedatives, e.g. cyclizine or prochlorperazine. Consider buccal/rectal routes of administration if vomiting. Do not use long-term • Encourage patients to mobilize after an acuteattack • Betahistine taken regularly may help in some patients, as may thiazide diuretics, a low-salt diet, vestibular rehabilitation, tinnitus maskers, and/ or hearingaids • There is some indication that stress may precipitate attacks • Look out for and treat concurrent anxiety and depression

Vertebro-basilar insufficiency Common in older patients. History of dizziness on extension and rotation of the neck. Normal tympanic membranes. May have associated cervical spondylosis and neck pain. Provide lifestyle advice. X Some advocate use of a cervical collar. Labyrinthectomy is a last resort and can help vertigo but results in deafness on that side. P. Ménière, (1799–1862)—French ENT surgeon.

Information and support for patients Action for Hearing Loss F 0808 808 0123 M www.rnid.org.uk British Tinnitus Association F 0800 018 0527 M www.tinnitus.org.uk Ménière’s Society F 0845 120 2975 M www.menieres.co.uk

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Ophthalmology ‘The eye is the window of themind’ Richard II, William Shakespeare (1564–1616) Assessment of the eye  954 Eye trauma  958 Eye pain, papilloedema, and orbital disease  960 Lid disease  962 Blepharitis and tear duct problems  964 The red eye and conjunctivitis  966 Corneal, sclera, and uveal disease  968 Visual field loss and blindness  970 Sudden loss of vision in one eye  972 Gradual loss of vision  974 Glaucoma  976 Cataract  978 Refraction errors and squint  980 Contact lenses and drugs for the eye  982

0 Community-based optometrists are a valuable resource available to GPs to help differentiate eye conditions and refine referral pathways. Getting an optometrist’s opinion can also assist in setting the appropriate priority level for referral or prevent unnecessary referrals.

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Assessment of theeye History  Ask about pain, redness, watering, change in appearance of the eye, altered vision, and if the problem is unilateral or bilateral. Distinguish between blurred and double vision. Enquire about trauma, previous similar episodes, systemic illness, and eye disease in the family. If using medication, take a drug history (including eye drops). Visual acuity  Test and record the central (macular) vision of each eye separately for near and distance vision with glasses on. Cover the non-test eye carefully. On a full-size Snellen chart, line ‘6’ can be read by the normal eye at 6m (see Figure 26.3—b p.957). If unable to read the 6/9 line, use a pinhole to improve refraction. Near vision can be checked using a near-vision testing card (see Figure 26.2—b p.956) or newspaper.

Examination • The eyelids should be symmetrical. Check the skin around the lids, the position, eyelashes of, and any inflammation, crusting, or swelling of the lid or lid margin • Use a bright light to examine the eye surface—it should be bright and shiny. Use a fluorescein stain if any indication of corneal damage • Note any redness—if most marked around the lid lining and periphery of the eye conjunctivitis is likely, whereas a duskier redness around the margin of the cornea (ciliary congestion) suggests disease of the cornea, iris, or deeper parts of the eye (uvea) Examining the ocular media  Takes practice. Darken the room and ensure you have good batteries in your ophthalmoscope. • Check the red reflex (opacities within the eye appear as a shadow) • Examine the disc—place your hand on the patient’s forehead and support the lid with your thumb; use your right eye for the patient’s right eye and vice versa, Look for the shape, colour and size of thecup • Follow each of the 4 main vessels to the periphery • Examine the macula by asking the patient to look directly at thelight • Examine the peripheral retina by asking the patient to look up,down 0 Dilating the pupils with a short-acting mydriatic (e.g. 0.5–1% tropicamide) makes examination much easier, but warn patients they may have temporarily blurred vision and should not drivehome. Visual fields  Test peripheral vision by sitting in front of the patient and comparing their visual field to your own (1 eye at a time)—the most basic test is to check the patient can see hand movement in each of the 4 quadrants. Refer for formal tests. Visual field defects— b p.971. Eye movements If the patient complains of double vision, move an object to the nine positions of gaze (see Figure 26.1). Ask the patient to tell you in which direction the double vision increases. Pupils  Should be round, central, of equal size, and respond equally to light and accommodation Pupil abnormalities: • Horner’s syndrome bp.300 • Fixed dilated pupil Causes: trauma (e.g. blow to the iris), mydriatic drops, acute glaucoma, third nervepalsy

Assessment of theeye

• Afferent pupillary defect Pupils are the same size but there is a d in constriction response to light in the affected eye. Shine a bright light in the better eye for 3s, then move it rapidly to the affected eye. If the afferent pathway is d, the first pupil movement is dilation not constriction. Causes: optic neuritis, retinal disease • Argyll Robertson pupil Bilateral small irregular pupils with no light response. Occurs in patients with DM or neurosyphilis. D. Argyll Robertson (1837–1909)—Scottish ophthalmologist • Holmes–Adie pupil Accommodation is partially paralysed, causing blurring of near vision, slight pupil dilation, and a very slow pupil response to light and accommodation (minutes). Occurs unilaterally in young adults and is not associated with serious neurological disease. G.M. Holmes (1876–1965)—Irish neurologist; W.J. Adie (1886–1935)—British neurologist Referral  See Table 26.1 Up Upper right

R

L

Upper left Left

Right Lower right

Lower left Down

Figure26.1  The nine positions of gaze (straight ahead is one position) Table26.1  Eye referrals Emergency (direct to A&E or emergency eye clinic)

Sudden loss of vision Acute glaucoma Perforating injury, intraocular foreignbody Chemicalburns Retinal detachment Cornealulcer Sudden onset of diplopia or squint+pain Temporal arteritis with visual symptoms—b p.524

Same-day (35mmHg) Chronic red eye conditions Painless diplopia orsquint Chalazion/stye/cyst Ptosis

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Figure26.2  Near-vision testing card—reading types are read at 30cm with reading glasses ifused Reproduced from Collier J, Longmore M, Amarakone K (2013) Oxford Handbook of Clinical Specialties, with permission from Oxford University Press.

Assessment of theeye

0 These Snellen charts are for illustration purposes only. Lines on a fullsized chart are read from a distance of 6m with distance glasses if worn. Read from the top of the chart to the bottom. Interpretation: Able to read down to the line labelled:

6

Normal vision

6/6

9

Can see at 6m what a normal person can see at 9m

6/9

12 Can see at 6m what a normal person can see at 12m 6/12 18 Can see at 6m what a normal person can see at 18m 6/18 36 Can see at 6m what a normal person can see at 36m 6/36 60 Can see at 6m what a normal person can see at 60m 6/60

Counts fingers

Counts fingers held at 0.5m distance

CF

Hand movement

Perceives hand moving at 0.25m distance

HM

Perceives light

Can see a torchlight when shone into the eye

PL

No perceived light

Blind

No PL

Figure26.3  Snellencharts Reproduced from Collier J, Longmore M, Amarakone K (2013) Oxford Handbook of Clinical Specialties, with permission from Oxford University Press.

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Eyetrauma In allcases • Take a careful history; establish the nature of the trauma (i.e. what hit the eye and with what force?) • Measure acuity and examine both eyes carefully recording your findings • If the patient is unable to open the injured eye, try to instill local anaesthetic drops and then examine—if unable to do so, refer to eye casualty for assessment • Encourage accident prevention, e.g. wearing protective goggles

Corneal abrasion • Take a careful history to exclude high-speed particles, (e.g. from strimmer) that could cause penetrating injury • Abrasions may cause severe pain—if so apply a few drops of local anaesthetic (e.g. proxymetacaine 0.5%) before examining • Use fluorescein stain, with cobalt blue light illumination to detect abrasion—stains green (see Figure26.4) • If the abrasion is vertical, ensure no foreign body is left in the eye by everting the upperlid • Abrasions normally heal in 12h, a rust ring may form around a metal foreign body—refer to eye casualty for removal Arc eye  Due to corneal epithelial damage as a result of exposure to UV light. Seen in welders, sunbed users, skiers, mountaineers, and sailors who do not use adequate eye protection. Symptoms include severe eye pain, watering, and blepharospasm a few hours after exposure. Management  Pad the eye and give analgesics and cyclopentolate 1% eye drops bd (causes pupil dilation). Recovery should occur in 40y to have regular optometry check-ups. Those with a family history of glaucoma should have biannual checks of their intraocular pressures (tonometry) and annual visual field checks at an optician from 40y of age. Refer patients with i pressures, or in whom you notice (or are doubtful about) disc cupping to ophthalmology for assessment. Patients with i intraocular pressure must be followed up life-long. Aim is to d intraocular pressure to slow disease progression (even with ‘normal’ pressures). Medical treatment • Topical prostaglandin analogue (e.g. latanoprost od in the evening)— i outflow of aqueous • Topical β-blocker (e.g. timolol 0.25% bd)—d aqueous secretion. Caution in patients with asthma or heart failure. Often combined with a topical prostaglandin analogue. Side effects: allergy and dryeyes • Topical carbonic anhydrase inhibitor (e.g. dorzolamide tds, or bd if in combination with a β-blocker)—d aqueous secretion. Side effects: blurred vision, tiredness, dyspepsia • Topical α-agonist (e.g. brimonidine bd)—d aqueous secretion and i outflow. Side effects: local reactions, headache, dry mouth, tiredness Surgery  Trabeculectomy is considered when the ‘target’ intraocular pressure is not met with medical treatment (especially in patients 40y—typically elderly, long-sighted women with early cataract. Closed-angle glaucoma may present in one of 3ways: • Latent Usually picked up when screening the opposite eye after an episode of acute/subacute glaucoma. The patient is asymptomatic and IOP normal, but the anterior chamber is shallow with a narrowangle. • Subacute Episodic haloes around bright lights, impaired vision ± frontal headache/eye pain. Attacks are precipitated by the pupil dilating, e.g. at night or entering a darkened room, and relieved by sleep or entering a brighter environment. Examination between attacks is normal but during an attack, the pupil is semi-dilated and cornea slightly clouded. Patients with subacute glaucoma are at risk of an acute attack • Acute Blockage of aqueous drainage from the anterior chamber causes a sudden i in IOP from 15–20 to 60–70mmHg. There may be a history of previous subacute attacks. The patient complains of eye pain with acute loss of vision in 1 eye ± abdominal pain/nausea/vomiting Examination  Vision d; cornea looks hazy (due to oedema); pupil is fixed and dilated (often slightly oval in shape with long axis vertical); circumcorneal redness; eyeball feels hard (due to i pressure); poor fundal view ± cataract. Management  Refer acute or subacute glaucoma as an emergency to ophthalmology. Specialist treatment is with miosis to open drainage channels (e.g. pilocarpine 4% drops) and acetazolamide ± apraclonidine and/ or latanoprost drops to d aqueous production. Surgery or laser treatment (peripheral iridotomy) to allow free aqueous circulation is undertaken once intraocular pressure has been d. Patient may need prophylactic surgery on the contralateral eye to prevent AACG in that eye too. AACG may damage the trabecular meshwork and patients are at risk of developing chronic glaucoma following an attack. Regular check-ups are necessary. Neovascular or secondary glaucoma May occur in patients with diabetic retinopathy, central or branch retinal vein obstruction, or ocular ischaemia. Blood vessels grow across the iris and the iridiocorneal angle, preventing fluid drainage. Pressures can be very high (40–70mmHg) and the patient may suffer pain from corneal oedema. Treatment is surgical and, in severe cases, if the eye is blind, it is removed. Congenital glaucoma  1:10,000 live births. 4 > 5. Usually bilateral. Presents with irritation of the eye (watering, rubbing), photophobia, large eyes with large, fixed pupils ± cloudy cornea. Refer urgently for paediatric ophthalmic opinion. Surgery is needed to prevent blindness.

Further information NICE Diagnosis and management of chronic open-angle glaucoma and ocular hypertension (2009) M www.nice.org.uk

Information and support for patients International Glaucoma Association F 01233 648170 M www.glaucoma-association.com

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Cataract Lens opacity is found in 75% >65y olds. Most do not need treatment.

Risk factors for cataract • Oldage • DM • +ve family history • Prolonged steroid treatment • iBP • Excessive alcohol

• Smoking • Prenatal rubella/toxoplasma (congenital cataract) • Hypocalcaemia • Eyetrauma • Radiation exposure

Presentation Inadults • Blurred vision and gradual loss ofvision • Dazzles and haloes around objects—especially in sunlight • Frequent spectacle changes due to changing refractiveindex 0 Unilateral cataract may not be noticed by the patient—but loss of binocular vision affects judgement of distance. In children  Cataracts present with squint, white pupil, nystagmus, amblyopia, or loss of binocular vision. May be hereditary or associated with Down’s syndrome, galactosaemia, or congenital rubella. Signs  A shadow in the red reflex/absent red reflex; difficulty visualizing the fundus.

Types of cataract • Congenital cataract—localized and usuallypolar • Nuclear cataracts—central; most common in oldage • Cortical lens opacities • Subcapsular cataracts—usually linked to old age or steroiduse. • Dot cataracts—commoninDM • Traumatic cataract      • Mature cataract (see Figure26.12)

Figure26.12  Mature cataract

Cataract

Management of adult cataract Check fasting blood glucose to exclude DM. Advise patients to have their visual acuity checked regularly. Refer to ophthalmology if d sight (or any other symptom) interferes with social functioning, driving, or independence. Surgical treatment  Removal of the natural lens ± posterior chamber lens implantation. Usually done as a day case procedure under LA. Healing takes 2–6wk depending on the technique used. 75–95% without other ocular pathology have 6/12 vision or better 3mo post-op. Patients require testing for new spectacles 6wk post-op to allow refractive changes to settle. Complications of cataract surgery • Intraocular infection (endophthalmitis)—rare (0.1%); presents with pain and blurred vision ± red eye ± tenderness. Refer back to the operating surgeon urgently—antibiotics injected within 2–3h can preserve vision. Delayed referral (>12h) will lead to blindness • Posterior capsule rupture • Broken or protruding sutures—cause sensation of a foreign body on the cornea or pain—may need to be removed • Vitreous haemorrhage • Glaucoma • Posterior capsule opacification (5–30% 7–8y of age, it becomes irreversible. Treatment is with glasses ± patching, and squint surgery if necessary.

Refractionerrors

Hypermetropia (long sight) Most common refractive error. Common in infants and lessens with age. Distant objects focus behind the retina. Ciliary muscle contraction (to make the lens more convex) is needed to focus the image. This can lead to convergent squint, eye tiredness, and headache. Convex lenses are used for correction. Myopia (short sight)  Distant objects focus in front of the retina. There is often a +ve FH. Concave lenses are used to correct the defect. Contact lenses may be necessary in high myopia (>–8 diopters). Myopia is unusual 1 appointment to achieve): • Establish a constructive relationship to enable effective two-way communication and serveas the basis for subsequent encounters • Assess the patient’s emotions and attitudes • Perform an assessment of the patient’s risk to self and/or others • Determine if the patienthasa mental disorder and, if so,which • Find out (where possible)what caused the mental disorder • Explore how best it mightbe treated

Psychiatric history Informants  GPs may be approached by concerned relatives/friends. Talk to them (be careful to maintain confidentiality); establish concerns/ circ*mstances, relevant past history, and outlinethe patient’s pre-morbid personality. The consultation  Try to review old notes before seeing the patient. Use open questions at the start, becoming directive when necessary—clarify, reflect, facilitate, listen. Be open and ready to ask about suicide, sex, drugs, etc. Askabout: • Presenting complaint—chronological account, past history of similar symptoms. Ask directly about thoughts of suicide and self-harm • Family history—psychiatric illness, recent loss or serious illnessofa family member, bereavement, depression, suicide or attempted suicide, psychosis, alcoholism, druguse • Personal history—abuse (as a child, domestic violence), substance misuse, serious illness (including past psychiatric history and major physical illness), recent significant events (e.g. childbirth, housemove) • Attitudesand beliefs—how does the patient see him/herself? What does he/shethink is wrong? How does he/she think other people view the situation? What doesthe patient want you to do aboutit? • Occupation—unemployed? Happy injob? • Home situation—housing, relationships, social support,debt etc.

Mentalstate examination  Check: • Appearance and behaviour—signsof self-neglect or malnutrition, eye contact, rapport, movements, agitation,or aggression • Speech—spontaneity, volume, tone, rate, amount, continuity (flightof ideas, loosening of associations) • Mood—depressed or elevated. Consider screening for depression, e.g. screening questions for patientswith chronic disease (b p.199), screening questions for pregnant or post-natal women (b p.839), PHQ-9(b p.1001) • Thinking—form, content, flow, possession • Perception—illusions, hallucinations, pseudohallucinations • Cognition—cognitive screen,e.g. 6CIT (b p.1011) • Insight—patient’s understanding of his/her illness, its effects, and needfor treatment

Mental health assessment

Action • Summarize the history back tothe patient and give an opportunity for him/her to fill in any gaps or clarifyany points • Drawupa problem list and management plan with the patient • Set a reviewdate

High-risk groups for psychiatric illness Women • More vulnerable to depression and eating disorders • During pregnancy and in the post-partumperiod • When looking after children 50% of refugees have mental disorders). Carers  All carers are at risk of depression (~40% of carers of stroke victims are depressed). This starts early after the onset of care-giving. It is good practiceto: • Identify all carers and mark their records • Check carers’ mental and physical health annually • Inform carers that they are entitled to a needs assessment • Ask patients if you can share information with theircarers • Inform carers about support groups and carer centres Residents of care homes and nursing homes  50% have depression.

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Mental health symptomsand signs Acute confusion  b p.1010 Anxiety bp.992 Depression  b p.1000 Abnormal beliefs  Decide whether a belief is normal inthe context of

the patient. If not, decide if the beliefisa: • Delusion, i.e. a beliefthat does not seem to have a rational basis and which is not amenable to argument, or • Overvalued idea, i.e. belief that is odd but understandable, given the patient’s background

Compulsions Forced behaviours, repeated despite inappropriateness or unreasonableness, and associated discomfortin response to an obsession. Can be disabling, e.g. repeated hand washing hundreds of times a day. Obsessive–compulsive disorder—bp.996

Abnormal perceptions  Consider: • Illusion Misinterpretationof information, e.g. seeing a coat on a hanger and interpreting it as a person.Can happen if d level of conscsiousness or occasionally if visual impairment—particularlyAMD • Hallucination • Pseudohallucination Vivid perception which is recognized as not being real, e.g. delirium tremens • Depersonalization Feeling of being unreal—like an actor playing yourself. Associated with a wide range of mental illness, e.g. depression, schizophrenia • Derealization Feeling of everything around you being unreal—like in a dream. Often linked to depersonalization Hallucinations  Sensory experiences in the absence of external stimuli. May be visual, auditory, gustatory, olfactory, or tactile. • Visual/tactile/auditory hallucinations Suggest mental illness • Visual and tactile hallucinations suggest organic disorder, e.g. dementia, acute delirium, metabolic encephalopathy, drugabuse • Auditory hallucinations suggest psychosis • Hallucinations experienced when the patient is falling asleep (hypnagogic hallucination) or waking up (hypnapompic hallucination) are features of narcolepsy • Olfactory and gustatory hallucinations Often occur together. May be suggestive of psychosis but also occur with temporal lobe epilepsy and olfactory bulb tumours Thought disorders  Consider disordersof: Content • Ideas of reference Patients feel they are noticed by everyone around them/stand out from the crowd; media content, e.g. television or radio, refers to the patient; or that others are talking/thinking about the patient. Becomes a delusion of reference when insight is lost. Associated with schizophrenia, depressive states, and acute and chronic cognitive impairment • Delusions

Mental health symptomsand signs

Flow • Flight of ideas Leaps from idea to idea. There is always some association between ideas but may seem odd, e.g. rhymes. Associated with manic illness • Perseveration Persistence of a verbal or other behaviour beyond what is apparently intended, expected, or needed. Associated with dementia and brain damage, e.g. cerebral palsy,CVA • Loosening of association Series of thoughts appear only distantly (orloosely) related to one another or completely unrelated. Associated with schizophrenia • Thought block Abrupt and complete interruption in the stream of thought leaving a blank mind. Associated with schizophrenia Form • Preoccupation The patient thinks about a topic frequently but can terminate the thoughts voluntarily. Common symptom, e.g. in anxiety states. Ask about preoccupation with suicide in depressed patients • Obsession Thought or image repeated in spite of its inappropriateness or intrusiveness and associated discomfort. The thought and efforts to stop it i anxiety and can be disabling Possession • Thought insertion Thoughts do not belong to the patient but have been planted there by someone else. One of the first-rank symptoms of schizophrenia • Thought withdrawal Opposite of thought insertion. The patient perceives a thought is missing and has been removed by someone else. Afirst-rank symptom of schizophrenia • Thought broadcasting The patient believes his/her thoughts can be heard by other people—either directly or via the newspapers, radio, etc. Associated with schizophrenia

Delusions Beliefs held unshakably despite available counter-evidence and which are unexpected in view of circ*mstances and background. The belief is usually (but not always)false. • Primary delusions Belief arrives in the head fully formed, e.g. thought insertion; strongly suggestive of schizophrenia • Secondary delusions Belief arises on the basis of experience, e.g. someone who has lost their job several times through no fault of their own may believe they are unemployable

Paranoid delusions  Delusions (usually primary) which concern the relationship between the patient and other people. Associated with schizophrenia, depressive states, and acute and chronic cognitive impairment. • Delusions of persecution Most common type of paranoid delusion. Belief that a person or an organization is intentionally harassing or inflicting harm upon the patient. Associated with schizophrenia, depressive states, and acute and chronic cognitive impairment • Delusions of grandeur Beliefs of possessing exaggerated power, importance, knowledge, or ability. Associated with mania and schizophrenia

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Psychological therapies In the UK, the Improving Access to Psychological Therapies (IAPT) programme in England and similar programmes in other areas of the UK have dramatically i access to psychological therapies. These may be used alone or in combination with drug therapies.

Who should be referred?  Consider referral for patientswith: • Depression • Stress • Phobias • Grief reactions • Somatization • Eating disorders • Obsessive– • Body dysmorphic compulsive disorder disorder • Medically unexplained symptoms

• Anxiety • Panic disorder • Personality disorders • Post-traumatic stress disorder

Problem-solving therapy (PST)  Can be an effective tool for use in the GP surgeryM. Involves drawing up a list of problems and generating and agreeing solutions, broken down into steps, for patients to work on as homework between sessions—b p.1003 Cognitive behaviour therapy(CBT) • Behavioural therapies Aim to change behaviour. Usually the therapist uses a system of graded exposure (systematic desensitization), combined with teaching a method of anxiety reduction • Cognitive therapy Focusses on people’s thoughts and the reasoning behind their assumptions on the basis that incorrect assumptions (that are often unconscious) l abnormal reactions which then reinforce these assumptions further (a vicious cycle) The patient learns to recognize negative or unhelpful thinking patterns. By enabling the patient to be more aware of this and teaching ways that the patient can challenge cognitive errors, more helpful thinking styles can result. Patients must then practice re-evaluating their thoughts and associated behaviours. At least as effective as drug treatmentM.

Individual non-facilitated self-help Computerized CBT (CCBT) Particularly useful for patients with mild symptoms or who do not wish to be referred to specialist psychological therapy services. • Free online interactive CCBT,e.g: • Living Life to the Full M www.livinglifetothefull.com • The Mood Gym and e-Couch M www.moodgym.anu.edu.au/welcome • Other computerized CBT, e.g. Beating the Blues. Afee is payable, but some PCOs have purchased this program for use by patients free of charge in GP surgeries M www.beatingtheblues.co.uk Bibliotherapy  For patients who are not computer-literate, self-help books based on CBT techniques are an alternative,e.g.: • Gilbert P (2000) Overcoming Depression. London: Constable & Robinson. ISBN:1841191256 • Burns D (2000) Feeling Good: The New Mood Therapy. London: Harper Collins. ISBN:9780380810338

Psychological therapies

Guided self-help Based on a CBT approach and available through psychological therapy services. Helpful for patients with mild symptoms. Uses books/printed materials under the supervision of a trained facilitator who introduces, monitors, then reviews the outcome of each treatment. Usually there is minimal contact with the facilitator(50% in the first year after treatment. 8wk group programme aimed at patients who have ≥3 depression relapses, with 4 further follow-up sessions in the year following therapy. Behavioural activation Therapist and patient work together, with the aim of identifying effects that the patient’s behaviour might have on symptoms, mood, and problems. Then they address any problematic behaviours. Techniques may include reducing avoidance, activity scheduling, graded exposure, and initiating positively reinforced behaviours. Interpersonal therapy (IPT) Individual or group therapy concentrating on the difficulties that arise in maintaining relationships with others. Focusses on current, not past, relationships and works on the premise that if interpersonal conflicts are resolved, both relationships and mood will improve. Useful for patients who can identify relationship difficulties. Psychoeducational groups  Group therapy can be used to explore depression or chronic physical health conditions, e.g. diabetes. Run by trained practitioners, they also involve the element of peer support. Applied relaxation  Group or individual therapy that teaches patients to relax quickly in different situations. Establishes the cues that herald feelings of anxiety and helps the patient to relax when those cues are felt. Effective treatment for anxiety statesM. Counselling  Usually reflective listening to encourage patients to think about and try to resolve their own difficulties. There is little evidence of beneficial effects or cost-effectivenessN, but if there is a specific, identifiable cause for the patient’s symptoms, counselling directed at the cause may be helpful,e.g.: • Relationship breakdown—counselling is available through organizations, such as RELATE (M www.relate.org.uk) • Bereavement—counselling is available via organizations, such as CRUSE (F 0870 167 1677; M www.cruse.org.uk) • Debt—counselling is available from the Citizens Advice Bureau, National Debtline (F 0808 808 4000), or the Consumer Credit Counselling Service Debt Remedy website (M www.stepchange.org) Further information NICE M www.nice.org.uk • Depression(2009) • Generalised anxiety disorder and panic disorder in adults(2011) • Common mental health disorders(2011) SIGN Non-pharmaceutical management of depression in adults:a national clinical guideline (2010). M www.sign.ac.uk

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Anxiety Anxiety is only considered abnormal when it occurs in the absence of a stressful trigger, impairs physical, occupational, or social functioning, and/ or is excessively severe or prolonged. Features include: Physical symptoms Psychological symptoms • Drymouth  • Paraesthesiae • Fearful anticipation • Tremor    • Tinnitus • Irritability • Dizziness   • Headache • Sensitivity tonoise • Epigastric discomfort • Restlessness • Difficulty swallowing • Poor concentration • Frequent/loose motions/flatulence • Worrying thoughts • Chest discomfort/constriction • Insomnia and/or nightmares • Difficulty breathing/hyperventilation • Depression • Palpitations/awareness of missedbeats • Obsessions • Frequency/urgency of micturition • Depersonalization • Sexual dysfunction • Fear of losing control/dying • Menstrual problems

Generalized anxiety disorder (GAD) Long-term condition, fluctuating in severity and nature, often beginning in adolescence. Lifetime prevalence ~5%. Key features are excessive, difficult-to-control worry about a number of events/activities occurring on most days for ≥6mo. Consider if history of anxiety/worry or if frequent attender with chronic physical health problem or no physical health problem but needing reassurance about somatic symptoms or repeated worrying about a wide range of different issues. Associations • Anxiety is often accompanied by depression (b p.1000) and may be a feature of early schizophrenia • Other conditions which can cause anxiety and/or mimic symptoms of anxiety include drug/alcohol use or withdrawal, caffeine abuse, thyrotoxicosis, hypoglycaemia, temporal lobe epilepsy, phaeochromocytoma Assessment  Check TFTs. Use GAD-2 score (see Box 27.1) for screening: • If score �3, consider anxiety disorder • If score 1wk to work and of possible side effects (short-term i in anxiety; GI symptoms). If >60y or other risk factors for GI bleeding, consider co-prescribingaPPI • Follow-up every 2–4wk in the first 3mo then every3mo • If drug treatment is effective, continue for>1y • If no benefit, consider alternative SSRI/SNRI or adding a psychological therapy; pregabalin is an option if unable to tolerate SSRI/SNRI. Do not offer antipsychotic medication • Avoid benzodiazepines except for acute crises; restrict use to single > • History of suicide attempt/ self-harm married • Alcohol/drugmisuse • Unemployment • Family history of suicide • Chronic physical illness Cultural considerations Some cultures have no terms for depression. Patients may present with physical symptoms (somatization) or use less familiar ‘cultural-specific’ terms to describe depressive symptoms, e.g. ‘sorrow in my heart’. Management of depressionN  Use a stepped care approach starting at the step most appropriate for the patient. Step 1  All patients—assess severity. Provide education about depression and information about support available to both the patient and carers/family members as appropriate. Discuss treatment options. If sleep is a problem  Discuss sleep hygiene (b p.194):establish regular sleep/wake times; avoid excess eating, smoking, or alcohol before sleep; create a proper environment for sleep; take regular exercise. Subthreshold or mild depression Active monitoring—watchful waiting for 2y; subthreshold/mild depression that persists after other interventions Step 3 Moderate/severe depression or mild/subthreshold depression that has not responded to treatment. • High-intensity psychological interventions (b p.990) Individual or group-based CBT, interpersonal therapy, behavioural activation, behavioural couples therapy (if relationship difficulties) • Drug treatment UsuallySSRI • Combined treatments Antidepressant medication + high-intensity psychological intervention (CBT or interpersonal therapy) • Collaborative care For people with physical health problems. Case management supervised by a mental health professional providing education + psychological/pharmacological treatments with follow-up Step 4 Referral to psychiatry  U=Urgent; S=Soon; R=Routine • High suiciderisk—U • Severe self-neglect—U • Depression complicated by psychotic symptoms—U • Depression complicated by significant psychiatric co-morbidity or psychosocial factors—R/S • Inadequate response to multiple treatments—R Safety-netting  Follow-up if patients do not attend appointments. Give patient ± family/carers clear advice on what to do if the patient’s mood deteriorates and how to access urgent support, both in and out ofhours.

Further reading NICE M www.nice.org.uk • Depression(2009) • Depression in adults with a chronic physical health problem(2009)

Patient information and support Depression Alliance F 0845 123 2320 M www.depressionalliance.org Samaritans F 08457 909 090 M www.samaritans.org

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Drugs for treating depression (BNF4.3)

When should antidepressants be started?  Considerfor: • Patients with moderate/severe depression ± psychological therapy • Dysthymia (subthreshold depressive symptoms lasting>2y) • Mild depression if other treatment strategies have failed—bp.998 What should Itell the patient?  Giving patients information i compliance. When starting antidepressant drugs explain: • The reasons for prescribing • Timescale of action—unlikely to have any effect for 2wk; effects build up to maximum effect at 4–6wk,and • Likely side effects including possible exacerbation of anxiety in the first 2wk of treatment Which drugs are available?  The major groupsare: Selective serotonin re-uptake inhibitors (SSRIs)  (e.g. fluoxetine 20mg od; citalopram 20–40mg od; sertraline 50–150mg od). Usually first choice as less likely to be discontinued due to side effects and safer in overdose. Warn of possible short-term i in anxiety/agitation when starting medication and advise patients to stop if significant. GI side effects, including dyspepsia are common. Consider co-prescribing a PPI for stomach protection if >60y or other risk factors for GI bleeding. Only fluoxetine has been shown to be of benefit for the treatment of depression in children—b p.921. E lderly people—particularly those taking SSRIs—are prone to hyponatraemia when taking antidepressants. Serotonin and noradrenaline re-uptake inhibitors (SNRIs)  (e.g. venlafaxine 37.5mg bd, duloxetine 60mg od). Avoid if uncontrolled hypertension. Venlafaxine is also contraindicated if high risk of arrhythmia. Tricyclic and related anti-depressants (TCAs)  (e.g. lofepramine 70mg od/ bd/tds; trazodone 150–300mg daily). Titrate dose up from low dose until the patient feels the drug is helping or until side effects intrudeS. Common side effects include drowsiness, dry mouth, blurred vision, constipation, urinary retention, and sweating. Use with caution for patients with CVD because of risk of arrhythmia, patients with prostatic hypertrophy (i risk of retention), and patients with raised intraocular pressures (i risk of acute glaucoma). Mirtazapine  15–45mg nocte. Presynaptic α2-adrenoreceptor antagonist. i central noradrenergic and serotonergic neurotransmission. Causes sedation during initial treatment and may also cause weighti. Reboxetine  4–6mg bd. Selective inhibitor of noradrenaline re-uptake. Not recommended for elderly patients.

Drugs for treating depression

Monoamine oxidase inhibitors (MAOIs) (e.g. phenelzine 15mg tds). Should only be initiated in a specialist setting. Do not startuntil: • >1–2wk after a tricyclic has been stopped (3wk in the case of clomipramine or imipramine) • >1wk after an SSRI has been stopped (2wk in the case of sertraline; 5wk in the case of fluoxetine) Patients taking MAOIs must be very careful with diet, eating only fresh foods and avoiding game, alcohol, and foods containing tyramine, such as mature cheese, pickled herring, broad bean pods, and meat, yeast, or soya bean extracts. Failure to do so can result in rapid i in BP (often heralded by a headache). 0 Do not start other antidepressants until 2wk after treatment with MAOIs has been stopped (3wk if starting clomipramine/imipramine).

Follow-up • Review patients every 1–2wk until stable assessing response, compliance, side effects, and suicidalrisk • Continue for 4–6wk before judging a treatment as failed—and a further 2–4wk if partial response • Continue treatment for at least 6mo in total—12mo in the elderly and those with generalized anxiety disorder. Advise patients with a history of recurrent depression to continue for>2yN Discontinuation reactions  Occur once a drug has been used ≥ 8wk. Usually become apparent 40y. 5:4 80.7. Drug management of cognitive symptoms Specialist-initiated—refer. Preferred drug varies according to severity of cognitive deficit: • Mild/moderate Anticholinesterase inhibitors (e.g. donepezil, galantamine, rivastigmine) d rate of decline. • Moderate/severe Memantine d clinical/cognitive decline Alois Alzheimer (1864–1915)—German neuropathologist/psychiatrist. Vascular (multi-infarct) dementia Multiple lacunar infarcts or larger strokes cause generalized intellectual impairment. Tends to occur in a stepwise progression with each subsequent infarct. The final picture is one of dementia, pseudobulbar palsy, and shuffling gait with small steps. Treatment is as for secondary prevention of TIA/stroke—b p.564 Lewy body dementia Fluctuating but persistent cognitive impairment, parkinsonism, and hallucinations. No specific treatment. Avoid antipsychotics as they can be fatal. Use benzodiazepines if tranquillization is necessary. Friedrich H.Lewy (1885–1950)—German neurologist. Pick’s dementia  Dementia characterized by personality change associated with frontal lobe signs such as gross tactlessness. Lack of restraint may lead to stealing, practical jokes, and unusual sexual adventures. Treatment is supportive. Arnold Pick (1851–1924)—Czech neurologist/psychiatrist.

Further information NICE Dementia (2006 with 2011 update) M www.nice.org.uk

Patient information and support Alzheimer’s Society F 0300 222 1122 M www.alzheimers.org.uk Benefits enquiry line F 08457 123 456 Carers UK F 0808 808 7777 M www.carersuk.org

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Eating disorders Identification of and screening for eating disorders Target groups for screening include: • Young women with low BMI compared with agenorms • Patients consulting with weight concerns who are not overweight • Women with menstrual disturbances or amenorrhoea • Patients with GI symptoms • Patients with symptoms/signs of starvation—sensitivity to cold, delayed gastric emptying, constipation, d BP, bradycardia, hypothermia • Patients with physical signs of repeated vomiting—pitted teeth 9 dental caries, general weakness, cardiac arrhythmias, renal damage, i risk of UTI, epileptic fits,dK+ • Children with poorgrowth • Young people with type 1 DM and poor treatment adherence Screen target populations with simple screening questionsN • Do you worry excessively about your weight? • Do you think you have an eating problem? • Patients who are pregnant or have DM are at particular risk of complications if they have eating disorders. Refer early for specialist support and ensure everyone involved in care is aware of the eating disorder.

Anorexia nervosa  Prevalence 0.02–0.04%. 5 >> 4. Usually begins in adolescence. Peak prevalence at 16–17y. Features: • Refusal to keep body weight >85% of that expected (BMI 4.Features: • Pervasive and maladaptive patterns of behaviour, thinking, and control of emotions • Significant instability of personal relationships, self-image, andmood • Impulsive behaviour • Tendency towards suicidal thoughts/self-harm • Possible transient psychotic symptoms

Recognition  Consider if repeated self-harm, persistent risk-taking, or marked emotional instability. If suspected, refer for specialist assessment. Crisis managementN • Assess current risk to self/others—if a risk, request emergency specialist assessment and/or acute admission voluntarily or under the Mental Health Act (b p.1122) • Ask about past episodes and management strategies thatworked • Help to identify manageable changes that might enable the patient to deal with current problems • Offer follow-up and contact the patient if the appointment is notkept • Refer for specialist care if risk/distress continuestoi Specialist treatment of personality disorder • Intensive structured psychological therapy, e.g. STEPPS programme • Drugs—SSRIs are used for impulsive behaviour; low-dose antipsychotics for paranoid ideas; mood stabilizers for emotional instability. 0 Be careful taking over prescribing from specialist care— there are no drugs licensed for use for borderline personality disorder and evidence of efficacy ispoor • Risk of overdose is i amongst patients with personality disorder.

Factitious disorder (Munchausen syndrome)  Intentional feigning of physical/psychological symptoms to assume the sick role (9 hospital admission). Can be difficult to detect. Differs from malingering as there is no external reward (e.g. financial). Common presentations • Physical Dermatitis artefacta, PUO, bruising disorders, brittle DM, diarrhoea of unknown cause; neurological symptoms, e.g. pseudoparalysis or pseudofits (neurologica diabolica); abdominal pain (laparotomophilia migrans); chest pain (cardiopathia fantastica)

Other psychological conditions

• Psychological Feigned psychosis, fictitious bereavement, fictitious overdose Management  Exclude any other basis for presenting pathology. Explain findings to the patient exploring possible causes. Assess psychological and social difficulties. Consider referral to specialist mental health services. Baron H.K.F.F.von Munchausen (1720–1797)—German traveller/soldier. Munchausen’s syndrome by proxy  Caregiver—typically a mother with child—seeks repeated medical investigations and needless treatment for the person he/she is caring for. The child or person being cared for may actually be harmed by the carer to achieve these aims. Commonly reported symptoms include:neurological symptoms, bleeding, rashes. Management  Often difficult to detect and even harder to prove. Aform of abuse that must be taken seriously and handled with care (b p.924). Involve all relevant agencies early (e.g. social services, paediatrics). Malingering  Intentional production or feigning of physical or psychological symptoms to assume the sick role for a known external purpose. Malingering is not considered a mental illness or psychopathology, although it can occur in the context of other mental illnesses.Forms: • Pure malingering The individual falsifies all symptoms • Partial malingering The individual has symptoms but exaggerates the impact they have upon daily functioning • Simulation The individual acts out symptoms of a specific disability • False attribution The individual has valid symptoms but is dishonest as to the source of the problems, e.g. attributing neck pain to an RTA to obtain compensation Differential diagnosis  True medical or psychiatric illness yet to be diagnosed; factitious disorder; somatization disorder Common motivating factors • Avoidance of work and/or other • Obtaining narcotics • Avoidance of/release fromjail responsibility • Need for attention • Litigation to obtainmoney Management  Difficult. As doctors, we tend to believe our patients. • Exclude causes for symptoms through careful history/examination • Avoid prescribing drugs and unnecessary referrals as these might perpetuate symptoms • Avoid certifying the patient as unfit to work or to perform activities— if the patient is unhappy about this, suggest a second opinion • Tactfully explain your findings and conclusions to the patient and explore the reasons for the behaviour • Provide support to find more appropriate ways to solve problems

Further information NICE Borderline personality disorder (2009) M www.nice.org.uk

Patient/relative information and support Emergence M www.emergenceplus.org.uk Borderline Personality Disorder (BPD) central M www.bpdcentral.com Self Injury and Related Issues (SIARI) M www.siari.co.uk 

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Cancer and palliativecare Principles of cancer care  1020 Surgery for cancer  1022 Chemotherapy  1024 Radiotherapy  1026 Palliative care in general practice  1028 Pain and general debility  1030 Anorexia, nausea, and vomiting  1032 Other GI problems  1034 Skin, neurological, and orthopaedic problems  1036 Respiratory problems  1038 Haematological and vascular problems  1040 Problems with mental well-being  1042 The last 48 hours  1044 Syringe drivers  1046

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Principles of cancercare If cancer is suspected, comprehensive assessment of patient and disease is needed before treatment decisions are made. Treatment canbe: • Radical Curative intent—surgery and/or drug/radiotherapy • Adjuvant Given after surgery when micrometastatic disease is suspected—decision to proceed is based on the likelihood of relapse • Neoadjuvant Given prior to definitive treatment to make treatment easier and more likely to succeed • Palliative When cure is not possible and symptom management is the priority—b p.1028

Assessment of thetumour • Histological nature of the tumour Tissue of origin; cancer type (e.g. adenocarcinoma, squamous cell cancer); degree of differentiation; and tumour grade. High-grade, poorly differentiated tumours tend to have a poorer outcome than low-grade, well-differentiated tumours • Biological behaviour of the tumour Tumour markers produced by cancers may be a useful adjunct to histological classification and staging and can be used to influence and monitor efficacy of treatment—see Table 28.1. 0 Tumour markers can be i in non-malignant conditions • Anatomical extent of the tumour Determined through clinical, radiological, biochemical, and surgical assessment. Routine blood tests (e.g. LFTs, bone profile) may also indicate the presence of metastases

Cancer staging  Staging allows the plan of treatment to bemade. TMN classification  Widely used classification of tumours. Exact criteria for staging depend on the primary organsite: • T Primary tumour—graded T1–T4 with increasing size of primary • N Regional lymph nodes—advancing nodal disease is gradedN0–N3 • M Presence (M1) or absence (M0) of metastases Stage grouping • Stage 1 Clinical examination reveals a tumour confined to the primary organ. The lesion tends to be operable and completely resectable • Stage 2 Clinical examination shows evidence of local spread into surrounding tissue and first draining LNs. The lesion is operable and resectable but there is a higher risk of further spread of disease • Stage 3 Clinical examination reveals extensive primary tumour with fixation to deeper structures and local invasion. The lesion may not be operable and may require a combination of treatment modalities • Stage 4 Evidence of distant metastases beyond the site of origin. The primary site may be surgically inoperable

Other factors • Patient’s performance status The Eastern Cooperative Oncology Group (ECOG) Performance Status Scale is widely used (see Table 28.2). Patients with ECOG score >2 are usually deemed unsuitable for most chemotherapy interventions • Mortality, morbidity, and efficacy of the procedure • Patient preferences

Principles of cancercare

Table28.1  Tumour markers and associated conditions Tumour marker

Associated conditions Malignant conditions Non-malignant conditions

CEA

GI tract cancers (particularly colorectal cancer)

Cirrhosis Pancreatitis Smoking

CA 19–9

Colorectal cancer Pancreatic cancer

Cholestasis

CA 125

Ovarian cancer Breastcancer Hepatocellular cancer

Cirrhosis Pregnancy Peritonitis Endometriosis

αFP

Hepatocellular cancer Germ cell cancers (not pure seminoma)

Liver disease—hepatitis/ cirrhosis Pregnancy Open neural tube defects

HCG

Germ cell cancers Choriocarcinoma and hydatidiform mole

Pregnancy

PSA

Prostate cancer

BPH Prostatitis Prostate instrumentation Acute urinary retention Physical exercise Old age

Table28.2  ECOG Performance StatusScale Classification

Description

ECOG 0 ECOG 1

Fully active; able to carry on all activities without restriction Restricted in physically strenuous activity but ambulatory and able to carry out work of a light or sedentary nature Ambulatory and capable of all self-care; confined to bed or chair 50% of waking hours Capable of only limited self care; confined to bed or chair 50% or more of waking hours Completely disabled; cannot carry out any self-care; totally confined to bed or chair

ECOG 2 ECOG 3 ECOG 4

The GP’s role Treatment for cancer is increasingly successful but also

increasingly complex. Treatment of cancer is largely a specialist activity, but the role of the GP is important at this time, even if mainly supportive. • Keep in touch with the family and up to date with treatment—provide support (e.g. advice on benefits/local services), preventive care (e.g. flu vaccination for patients and/or carers), and general medicalcare • Liaise with the secondary care teams involved, and provide continuity if care is passed from one specialist team to another • If the patient does not survive, provide ongoing support to the family

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Surgery forcancer Surgery has 3 main roles in cancer management: Diagnosis and staging Advances in imaging and laparoscopic techniques have d the number of patients requiring open surgery to confirm a cancer diagnosis. Surgical staging remains importantin: • Breast cancer—‘sentinel’ axillary node biopsy is needed to accurately predict the state of nodal disease • Ovarian cancer—tumour deposits on the peritoneal surface are poorly visualized with conventional imaging. Direct visualization is required using laparotomy or laparoscopy • Certain abdominal malignancies—laparoscopic assessment of extent and spread of tumour may be performed prior to resection

Curative surgery Non-metastatic disease Surgery with curative intent is dependent on complete resection of the tumour with a margin of normal tissue. Local control of tumours with a propensity to spread to lymph nodes may be improved with resection of the draining group of nodes, e.g. vulval tumours. However, even if the tumour was completely resected, surgery can still fail to cure either as a resultof: • Development of metastatic disease as a result of the presence of micrometastatic deposits unidentifiable at the time of surgery • Development of local relapse as a result of inadequate margins. Surgical margins can be limited by patient-related factors (e.g. only a partial lobectomy may be possible in patients with lung cancer because of poor underlying respiratory function) or by tumour-related margins (e.g. invasion of the tumour to a vital structure such as theaorta) Metastatic disease Surgery may be curative in a limited number of tumours with metastases. However, this is much less common, requires careful patient selection, and is best performed by a specialist team. Circ*mstances in which curative surgery may be offered include: • Isolated metastases from breast cancer with a long disease-free interval • Liver metastases from colorectalcancer • Pulmonary metastases from osteosarcoma or soft tissue sarcoma Palliative surgery  Surgery can be effective in achieving good symptom control in the palliative setting, but decision to proceed must be carefully considered particularly as patients may have limited life expectancy, poor performance status, and rapid tumour progression. Ideally such decisions should be multidisciplinary and involve surgeons specialized in oncology and experienced in palliative management (see Table28.3).

Surgery forcancer

Table28.3  Situations in which palliative surgery should be considered Situation

Comments

Cancers causing obstructive symptoms e.g. bowel, ovary, ureter, bronchus 0 Most malignancyassociated bowel obstructions are functional, not anatomical

Surgery to relieve the obstruction may be warranted even if the underlying disease is incurable with locally advanced disease or distant metastases Bowel obstruction this occurs most commonly in patients with colonic or ovarian cancer Oesophageal or bronchial obstruction laser therapy of an intraluminal mass may restore thelumen Obstructive hydronephrosisnephrostomy or ureteric catheters may relieve the obstruction Placement of a stent may help relieve the symptoms of dysphagia, dyspnoea, jaundice, and large bowel obstruction

Fistulae

Fistulae, often arising as a result of pelvic tumours or as a side effect of radiotherapy, can be associated with distressing malodours and excessive discharge Surgery may provide excellent palliation but may not be useful in those with multiple sites of fistulae or rapidly advancing intra-abdominal disease where life expectancy is limited

Jaundice

Radiological and/or endoscopic stent placement: can relieve obstructive jaundice secondary to extrinsic pressure from lymph nodes on the biliary system or intrinsic pressure from cholangiocarcinoma or pancreatic carcinoma Complications infection or blockage necessitate replacement Surgical relief by choledochoenterostomy; avoids the problems associated with stents and may be indicated in a small minority with excellent performance status and slowly growing disease

Spinal cord compression and brain tumours

Urgent referral for neurological assessment for decompressive surgery or vertebroplasty is indicated for confirmed spinal disease or operable brain tumours

Gastrointestinal bleeding

A wide range of endoscopic techniques have been developed to stop bleeding from benign and malignant causes, including sclerotherapy with adrenaline, laser coagulation, and radiological embolization These techniques may avoid the need for major surgery in patients who have a limited life expectancy

Bone metastases

Prophylactic fixation of a long bone may reduce either pain and/or the risk of pathological fracture in patientswith: •  Lesions in weight-bearingbones •  Destruction of >50% of thecortex •  Pain on weight-bearing •  Lytic lesions In all cases fixation should be followed by radiotherapy to control growth and promote healing

Pain

If the expected morbidity of the procedure is low, surgical debulking of large, slowly growing tumours can reduce pain. Neurosurgical approaches such as cordotomy are only rarely considered

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Chemotherapy Chemotherapy is the use of chemical agents in the cure or palliation of malignant disease.

Drug groups Include:

• Antibiotics, e.g. bleomycin • Alkylating agents, e.g. busulfan • Antimetabolites, e.g. methotrexate, 5-fluorouracil • Alkaloids, e.g. vincristine • Platinum derivatives—DNA intercalating agents, e.g. cisplatin • Enzymes, e.g. asparaginase • Hormones, e.g. sex hormones, corticosteroids • Biological agents, e.g. interferon, monoclonal antibodies (e.g. rituximab) • Others, e.g. hydroxycarbamide, retinoids These agents work in a variety of ways to inhibit tumour growth and/or cause tumour cell damage. Normal cells may be damaged at the same time as tumour cells, resulting in the high levels of toxicity experienced by patients.

Choice of agent  Depends on known activity of the agent, cost, and patient factors. It is a specialist decision.

Types of tumour  Response to chemotherapy depends on type and

grade of tumour being treated. Broadly tumours can be dividedinto: • Those likely to respond Leukaemia, lymphoma (Hodgkin’s and intermediate-/high-grade non-Hodgkin’s), testicular tumours, small cell lung cancer, embryonal tumours, choriocarcinoma, ovarian cancer, sarcoma, breast cancer, prostatecancer • Those that may respond Low-grade non-Hodgkin’s lymphoma, GI cancer, brain/CNS tumours, melanoma, bladder and uterinecancer • Those unlikely to respond Non-small cell lung, renal, pancreatic, head and neck, cervical, and livercancer

Combination chemotherapy Often different chemotherapeutic agents are combined to i their chances of effect. Agents acting in different ways may potentiate each others’ actions, and using combinations reduces the risk of resistance (if one agent does not have any effect, another may). Choosing agents with different side effect profiles reduces cumulative toxic effects. Intermittent chemotherapy  Particularly useful for cytotoxic drugs. Intermittent treatment exploits the difference in recovery rates between normal and malignant tissues. Gaps between cycles of treatment allow normal tissue (particularly the immune system) to recover, but the malignant tissue does not recover to such a large extent (see Figure 28.1). The population of malignant cells diminishes relative to the normal cells with eachcycle. Adjuvant chemotherapy Given to prevent relapse after primary

treatment of a non-metastatic tumour for which relapse rate is known to be high. An example is adjuvant chemotherapy for breast cancer.

Chemotherapy

Chemotherapy Normal cells Cancer cells Number of cells Time

Figure28.1  Action of cytotoxic chemotherapy on normal and cancer cell

populations

Neutropenic sepsis  Neutropenic sepsis is defined as fever of ≥38.0ºC for ≥2h when the neutrophil count is 12mo after treatment

Cerebral oedema

Can occur after cranial irradiation. Steroid dose is d after completion of radiotherapy. Consider i dose again

Memory loss

Depending on the parts of the cranium irradiated, both longand short-term memory problems can occur after cranial irradiation.Treatment is supportive; some recovery may occur

Somnolence syndrome

Occurs within a few weeks of brain irradiation. Presents with: nausea/ vomiting; anorexia; dysarthria; ataxia; profound lethargy Treatment is supportive. Recovery may occur spontaneously

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Palliative care in general practice ‘Any man’s death diminishes me because Iam involved in mankind’ Devotions Meditation 17, John Donne (1572–1631) • Death is the natural end to life—not a failure of medicine. Palliative care starts when the emphasis changes from curing disease and prolonging life to relieving symptoms and maintaining well-being or ‘quality of life’. On average, GPs have 1–2 patients with terminal disease at any time and can get more personally involved with them than other patients.

End-of-life care (EOLC)  75% of deaths are ‘predictable’ and follow a period of chronic illness where end-of-life care (for those likely to die in 30%) and breast cancer(40%). Presentation and differential diagnosis  bp.366 • Always suspect hypercalcaemia if someone is iller than expected for no obvious reason. Untreated hypercalcaemia can befatal. Management  Depending on the general state of the patient, make a decision whether to treat the hypercalcaemia or not. If a decision is made not to treat, provide symptom control and do not check the serum calcium again. If you decide totreat: • Asymptomatic patient with corrected calcium 3mmol/L Arrange treatment with IV fluids and bisphosphonates via oncologist/ palliative care team immediately. Check serum calcium 7–10d post-treatment. 20% do not respond, and there is no benefit from re-treating with the same bisphosphonate. Zoledronic acid, although more expensive than pamidronate, works for 72x as long (6wk). Consider maintenance with regular IV bisphosphonate. Many initially responsive to bisphosphonates become unresponsive withtime; monthly sc denosumab is an option for those with persistent/relapsed hypercalcaemia of malignancy

Pain and general debility

Table28.6  Management of specific types ofpain Type of pain

Management

Bone pain

•  Try NSAIDs and/or strong opioids • Consider referral for palliative radiotherapy, strontium treatment (prostate cancer), or IV bisphosphonates (d pain in myeloma, breast and prostate cancer) • Refer to orthopaedics if any lytic metastases at risk of fracture for consideration of pinning

Abdominal pain

•  Constipation is the most common cause; b p.1035 •  Colic try loperamide 2–4mg qds or hyoscine hydrobromide 300 micrograms tds s/ling. Hyoscine butylbromide (Buscopan®) 20–60mg/24h can also be given via syringe driver •  Liver capsule pain dexamethasone 4–8mg/d. Titrate dose to the minimum that controls pain. Alternatively try NSAID + PPIcover •  Gastric distension may be helped by an antacid 9 an anti-foaming agent (e.g. Asilone®). Alternatively a prokinetic may help, e.g. metoclopramide or domperidone 10mg tds beforemeals •  Upper GI tumour often neuropathic element of pain; coeliac plexus block may help; refer to the palliative careteam •  Consider drug causes NSAIDs are a common iatrogeniccause •  Acute/subacute obstruction b p.1033

Neuropathic pain (pain associated with altered sensation)

• Often burning/shooting pain; usually only partially responsive to opioids—titrate to the maximum tolerated dose ofopioid • If inadequate add a neuropathic agent, e.g. amitriptyline 10–25mg nocte, increasing as needed every 2wk to 75–150mg. Alternatives include gabapentin, pregabalin, duloxetine, and clonazepam • If pain is due to nerve compression resulting from tumour, dexamethasone 4–8mg od mayhelp • Other options: TENS; nerve block; topical lidocaine patches; specialist treatment options, e.g. ketamine (seek expert advice)

Rectal pain

•  Topical drugs, e.g. rectal steroids •  Tricyclic antidepressants, e.g. amitriptyline 10–100mgnocte •  Anal spasms—glyceryl trinitrate ointment 0.1–0.2%bd •  Referral for local radiotherapy

Muscle pain

•  Paracetamol and/orNSAIDs • Muscle relaxants, e.g. diazepam 5–10mg od, baclofen 5–10mg tds dantrolene 25mg od, increasing at weekly intervals to 75mgtds •  Physiotherapy, aromatherapy, relaxation, heat pads

Bladder pain/ spasm

•  Treat reversible causes. i fluids. Toilet regularly • Try oxybutynin 5mg tds, tolterodine 2mg bd, propiverine 15mg od/ bd/tds, or trospium 20mgbd •  Amitriptyline 10—75mg nocte is often effective • If catheterized—try instilling 20mL of intravesical bupivacaine 0.25% for 15min tds or oxybutynin 5mL in 30mL od/bd/tds •  NSAIDs can also beuseful • Steroids, e.g. dexamethasone 4–8mg od may d tumour related bladder inflammation • In the terminal situation hyoscine butylbromide 60–120mg/24h or glycopyrronium 0.4–0.8mg/24h sc can be helpful

Pain of short duration

For example, dressing changes—try a short-acting opioid e.g. fentanyl citrate 200 micrograms lozenge sucked for 15min prior to the procedure or a breakthrough dose of oral morphine 20min prior to the procedure

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Anorexia, nausea, and vomiting Anorexia Treat nausea, mouth problems, pain, and other symptoms.

d psychological distress and treat depression. Advise small, appetizing meals frequently in comfortable surroundings. Drugs that may be helpful • Alcohol pre-meals • Metoclopramide or domperidone 10mg tds pre-meals—to prevent feeling of satiety caused by gastricstasis • Dexamethasone 2–4mg od or prednisolone 15–30mg od for short-term appetite enhancement

General principles of management of nausea and vomiting • Assess Try to identify likely cause—see Table28.7 • Review medication Could medication be the cause? Which anti-emetics have been used before and how effective werethey? • Try non-drug measures • Choose an antiemetic If cause can be identified, choose an antiemetic appropriate for the cause (see Table 28.7). Use the antiemetic ladder (see Figure 28.3). Administer antiemetics regularly rather than prn and choose an appropriate route of administration • Review frequently—Is the antiemetic effective? Has the underlying cause of the nausea/vomiting resolved? Avoid changing antiemetic before it has been given an adequate trial at maximumdose 0 If there is >1 cause for nausea/vomiting, you may need >1drug. Route of administration • For prophylaxis of nausea and vomiting—use po medication • For established nausea or vomiting—consider a parenteral route e.g. syringe driver (b p.1046)—persistent nausea may d gastric emptying and drug absorption. Once symptoms are controlled consider reverting to a poroute Non-drug measures  Do not forget non-drug measures to d nausea: • Avoidance of food smells and unpleasantodours • Relaxation/diversion/anxiety management • Acupressure/acupuncture 2nd line narrow spectrum e.g. ondansetron Selected narrow spectrum OR broad spectrum e.g. levomepromazine antiemetic e.g. OR combination e.g. levomepromazine + —haloperidol or ondansetron —cyclizine or —metoclopramide STEP 2 STEP 1

± administer by syringe driver ± dexamethasone

Figure28.3  The antiemeticladder

Anorexia, nausea, and vomiting

Table28.7  Causes of vomiting and choice of antiemetic Mechanism of vomiting

Antiemetic

Drug/toxin-induced or metabolic, e.g. hypercalcaemia

Haloperidol (1.5–5mg nocte) Levomepromazine (5mg stat or 6.25mgnocte) If persistent nausea due to opioids, consider changing opioid

Chemotherapy/radiotherapy

Granisetron (1mg bd) or ondansetron (8mg bd po or 16mg od PR)—chemotherapy- or radiotherapy-induced vomiting Haloperidol 1.5–5mg nocte—radiotherapy-induced vomiting Dexamethasone 4–8mg daily po/sc—often given as part of a chemotherapyregime Metoclopramide 20mg tds

i intracranial pressure

Dexamethasone 4–16mg/d Cyclizine 50mg bd/tds (or 150mg/d via syringe driver)

Anxiety, fear, or pain

Benzodiazepines, e.g. diazepam 2–10mg/d or midazolam sc Cyclizine 50mgbd/tds Levomepromazine 6–25mg/d

Motion/position

Cyclizine 50mg tds po/sc/IM Hyoscine po (300 micrograms tds) or transdermally (1mg/72h) Prochlorperazine po (5mg qds) or buccal (3–6mg bd)

Gastric stasis*

Domperidone 10mg tds or metoclopramide 10mg tds (particularly if multifactorial with gastric stasis and a central component)

Gastric irritation

Stop the irritant if possible, e.g. stop NSAIDs Proton pump inhibitors, e.g. lansoprazole 30mg od or omeprazole 20mgod Antacids Misoprostol 200 micrograms bd—if caused by NSAIDs

Constipation

Laxatives/suppositories/enemas

Intestinal obstruction

Refer for surgery if appropriate Cyclizine, haloperidol, or levomepromazine Dexamethasone 4–8 mg/d—antiemetic and d obstruction If vomiting cannot be controlled consider referral for venting gastrostomy or antisecretory agents (e.g. octreotide)

Cough-induced

b p.1038

Unknown cause

Cyclizine 50mg tds or 150mg/d via syringe driver Levomepromazine 6–25mg/d Dexamethasone 4–8mg dailypo/sc Metoclopramide 10–20 mg tds/qds po

* Vomits of undigested food without nausea soon after eating.

0 Drugs with antimuscarinic effects (e.g. cyclizine) antagonize prokinetic drugs (e.g. metoclopramide)—if possible, do not use concurrently.

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Other GI problems Mouth problems  Review medication making the mouth sore or dry. Refer to the DN for advice on mouth care (e.g. use a toothbrush to keep the tongue clean). Consider mouthwashes, e.g. saline, Oraldene®, chlorhexidine, benzydamine (for pain). Try ¼–½ ascorbic acid 1g effervescent tablet/d— place on tongue and allow to dissolve. Specific measures • Oral thrush—treat with fluconazole 50mg od for 7d and soak dentures in sodium hypochlorite fluid for ≥12h to prevent reinfection • Painful mouth—benzydamine mouthwash 9 lidocainespray • Ulcers or painful areas—hydrocortisone pellets topically qds after eating andnocte • Oral cancer pain—topical NSAIDs, e.g. soluble aspirin or diclofenac • Chemotherapy-induced ulcers— sucralfate suspension • Dry mouth—review medication that might be causing dry mouth, e.g. antidepressants, opioids. Try salivary stimulants, e.g. iced water, pineapple chunks, chewing gum, boiled sweets, or mints. Consider saliva substitutes, e.g. Glandosane®spray • Radiotherapy-induced dryness—pilocarpine • Excessive salivation—amitriptyline 10–100mg nocte, hyoscine, or glycopyrronium via syringe driver Dysphagia May be due to physical obstruction (by tumour bulk) or functional obstruction (neurological deficit). • Treat the cause if possible, e.g. celestin tube for oesophagealtumour • If the patient is hungry and wishes to be fed consider referral for a percutaneous endoscopic gastrostomy(PEG) • If the patient does not wish to have a PEG ask whether he/she would like subcutaneous fluids and treat symptomatically with mouth care, anxiolytics, analgesia, and sedation Hiccup  A distressing symptom. Treatment is often unsatisfactory. • General measures Rebreathing with a paper bag; pharyngeal stimulation by drinking cold water or taking a teaspoon of granulatedsugar • Peripheral hiccups Irritation of the phrenic nerve or diaphragm—try metoclopramide (10mg tds), antacids containing simeticone (e.g. Asilone®), dexamethasone (4–12mg/d), or ranitidine (150mgbd) • Central hiccups Due to medullary stimulation, e.g. i ICP, uraemia— try chlorpromazine (10–25mg tds/qds), dexamethasone (4–12mg/d), nifedipine (10mg tds), or baclofen (5mgbd) Ascites  Free fluid in the peritoneal cavity. Common with ovarian cancer (50% patients). Presents with abdominal distension. Signs: shifting dullness to percussion 9 fluid thrill. Depending on clinical state consider referring for radio- or chemotherapy if appropriate. Symptom control • Give analgesia for discomfort • Refer for paracentesis and/or peritoneovenousshunt • Try diuretics—furosemide 20–40mg od and/or spironolactone 100–400mg od. May take a week to produce maximal effect. 0 Monitor albumin level—if low, diuretics make ascitesworse

Other GI problems

• Dexamethasone 2–4mg daily may help—discontinue if not effective • ‘Squashed stomach syndrome’—try prokinetics, e.g. domperidone or metoclopramide 10mgtds

Constipation  Passage of hard stools less frequently than the patient’s own normal pattern. It is a very common symptom. Occult presentations are common in the very elderly and frail and include: • Confusion • Abdominalpain • Loss of appetite • Urinary retention • Overflow diarrhoea • Nausea/vomiting • Constipation can herald spinal cord compression (b p.478). If suspected, do a full neurological examination. Management  Pre-empt constipation by putting everyone at risk (e.g. patients on opioids) on regular aperients. Treat reversible causes, e.g. give analgesia if pain on defecation, alter diet (e.g. add prunes), i fluid intake. • Treat with regular stool softener (e.g. lactulose, macrogol) 9 regular bowel stimulant (e.g. senna) or a combination drug (e.g. co-danthrusate). Titrate dose against response • If that is ineffective consider adding rectal measures. If soft stools and lax rectum—try bisacodyl suppositories (0 must come into direct contact with rectum); if hard stools—try glycerin suppositories; insert into the faeces, and allow to dissolve • If still not cleared refer to the district nurse for lubricant 9 stimulant enema (usually acts in 720min). Once cleared leave on a regular aperient, with instructions to i aperients if constipation recurs Gut fistulae Connections from the gut to other organs—commonly skin, bladder, or vagin*. Bowel fistulae are characterized by air passing through the fistula channel. If well enough for surgery, refer to a surgeon. If not fit for surgery consider referring to palliative care for octreotide. Diarrhoea Clarify what the patient/carer means by diarrhoea. Less common than constipation but can be distressing for the patient and difficult for the carer—especially if incontinence results. Management • i fluid intake—small amounts of clear fluids frequently • Screen for infection (including pseudomembranous colitis if diarrhoea after a course of antibiotics) and treat if necessary • Ensure no overflow diarrhoea s to constipation; no excessive/erratic laxative use; and no other medication is causing diarrhoea • Consider giving aspirin (300–600mg tds)—d intestinal electrolyte and water secretion caused by prostaglandins. May particularly help with radiation induced diarrhoea • Consider ondansetron 4mg tds for radiotherapy-induced diarrhoea • Consider giving pancreatic enzyme supplements, e.g. Creon® 25,000 tds prior to meals if fat malabsorption (e.g. s to pancreatic carcinoma) • Otherwise treat symptomatically with codeine phosphate 30–60mg qds or loperamide 2mg tds/qds • Refer to palliative care if unable to control symptoms

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Skin, neurological, and orthopaedic problems Bed sores  Due to pressure necrosis of the skin. Immobile patients are at high risk—especially if frail 9 incontinent. Likely sites of pressure damage—shoulder blades, elbows, spine, buttocks, knees, ankles, and heels. Bed sores heal slowly in terminally ill patients and are a source of discomfort and stress for both patients and carers (who often feel guilty that a pressure sore is a mark of poorcare). • If at risk refer to the DN or palliative care nursing team for advice on prevention of bed sores—protective mattresses and cushions, incontinence advice, advice on positioning and movement • Warn carers to make contact with the DN or palliative care nursing team if a red patch does not improve 24h after relieving the pressure on thearea • Treat any sores that develop aggressively and admit if not resolving Wound care  Large wounds can have major impact on quality oflife. Patients with advanced disease have major risk factors for development and poor healing of wounds—immobility, poor nutrition, skin infiltration ± breakdown due to malignancy. Skin infiltration causing ulceration or fungating wounds can be particularly distressing. Management  The primary aim is comfort. Healing is a secondary aim and may be impossible. Always involve the DN and/or specialist palliative care nursing team early. Many hospitals also have wound care specialist nurses who are valuable sources of advice. Specific management problems  See Table28.8.

Raised intracranial pressure  Occurs with p or s brain tumours.

Characterizedby • Headache—worse onlying • Vomiting

• Confusion • Diplopia

• Convulsions • Papilloedema

Management • Unless a terminal event, refer urgently to neurosurgery for assessment. Options include insertion of a shunt or cranial radiotherapy • If no further active treatment is appropriate start symptomatic treatment—raise the head of the bed, start dexamethasone 16mg/d (stop if no response in 1wk), analgesia

Spinal cord compression  bp.478 Bone fractures Common in advanced cancer due to osteoporosis,

trauma as a result of falls, or metastases. Have a low index of suspicion if a new bony pain develops. Treat with analgesia. Unless in a very terminal state, confirm the fracture on X-ray and refer to orthopaedics or radiotherapy urgently for consideration of fixation (long bones, wrist, neck of femur) and/or radiotherapy (rib fractures, vertebral fractures). In the elderly, fracture of a long bone can present as acute confusion.

Skin, neurological, and orthopaedic problems

Table28.8  Common wound management problems Problem

Management

Pain

Exclude infection; ensure the dressing is comfortable; limit frequency of dressing changes Ensure adequate background analgesia; consider additional analgesia for dressing changes and/or topical opioids on the dressing

Excessive exudate

Use high absorbency dressings with further packing on top ± plastic pads to protect clothing Change the top layer of the dressing as often as needed but avoid frequent changes of the dressing placed directly on thewound Protect the surrounding skin with a barrier cream/spray

Necrotic tissue

Use desloughing agents Referral for surgical debridement may be necessary

Bleeding

Prevent bleeding during dressing changesby: •  Avoiding frequent dressing changes • Using non-adherent dressings or dressings which liquefy and can be washed off (e.g. Sorbsan®)and •  Irrigating the wound with saline to remove dressings If there is surface bleeding—put pressure on the wound; if pressure is not workingtry: • Kaltostat® •  Adrenaline—1mg/mL (or 1:1,000) on a gauze pad,or • Sucralfate liquid—place on a non-adherent dressing and apply firmly to the bleedingarea Consider referral for radiotherapy or palliative surgery (e.g. cautery)

Odour

Treat with systemic and/or topical metronidazole Charcoal dressings can be helpful Seal the wound, e.g. with additional layer of cling film dressing Try disguising the smell with deodorizers (e.g. Nilodor®) used sparingly on top of the dressing—short-term measure. Long-term, the deodorant smell often becomes associated with the smell of the wound for the patient

Infection

Usually chronic and localized Irrigate the wound with warm saline or under running water in the shower/bath If the surrounding skin is inflamed—swab the wound and send for M,C&S then start oral antibiotics, e.g. flucloxacillin 250–500mg qds or erythromycin 250–500mg qds. Alter antibiotics depending on sensitivities of the organisms grown

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Respiratory problems Cough Troublesome symptom. Prolonged bouts of coughing are exhausting and frightening—especially if associated with breathlessness and/or haemoptysis. Haemoptysis  bp.297 Breathlessness  Affects 70% of terminally ill patients. It is usually multifactorial. Breathlessness always has a psychological element—being short of breath is frightening. Causes—see Figure28.4. Management of cough and breathlessness General non-drug measures • Generally reassure. Explain reasons for breathlessness/cough and adaptations to lifestyle that might help, e.g. sitting up straight • Breathing exercises can help—refer to physiotherapy • Exclude treatable causes (see Box 28.1 and Figure28.4) • Steam inhalations/nebulized saline can help with tenacious secretions • Try a stream of air over the face if the patient is breathless, e.g. fan, openwindow General drug measures • Try simple linctus 5–10mL prn forcough • Oral or subcutaneous opioids to d the subjective sensation of breathlessness—start with 2.5mg morphine sulfate solution 4-hourly and titrate upwards. Opioids may also help with cough—try pholcodine 10mL tds or morphine sulfate solution as for breathlessness. If already on opioids, i dose by 25%. Titrate dose until symptoms are controlled or side effects • Try benzodiazepines—2–5mg diazepam od/bd for associated anxiety + lorazepam 1–2mg s/ling prn in between. Diazepam acts as a central cough suppressant—try 2–10mg tds forcough • Oxygen has a variable effect and is worth a try, although a hand-held battery fan or electric fan may be just as effective • Hyoscine 400–600 micrograms 4–8-hourly (or 0.6–2.4mg/24h via syringe driver) and/or ipratropium inhalers/nebulized ipratropium d secretions Specific measures • Chest infection Treat with nebulized saline to make secretions less viscous 9 antibiotics (if not considered a terminalevent) • Post-nasal drip Steam inhalations, steroid nasal spray or drops 9 antibiotics • Laryngeal irritation Try inhaled steroids, e.g. Clenil® 100 micrograms/ actuation 2 puffsbd • Bronchospasm Try bronchodilators 9 inhaled or oral steroids. 0 Salbutamol may help cough even in the absence of wheeze • Gastric reflux Try antacids containing simeticone (e.g. Asilone®) • Lung cancer Try inhaled sodium cromoglicate 10mg qds; local anaesthesia using nebulized bupivacaine or lidocaine can be helpful— refer for specialist advice (avoid eating/drinking for 1h afterwards to avoid aspiration). Palliative radiotherapy or chemotherapy can also relieve cough in patients with lung cancer—refer

Respiratory problems

Stridor  Coarse wheezing sound that results from the obstruction of a major airway, e.g. larynx. Management • Corticosteroids (e.g. dexamethasone 16mg/d) can give relief • Consider referral for radiotherapy or endoscopic insertion of a stent if appropriate • If a terminal event—sedate with high doses of midazolam (10–40mg repeatedprn)

Box28.1  Reversible causes ofcough • Infection • Malignant bronchial obstruction/lung metastases • Bronchospasm • Gastro-oesophagealreflux •  Heart failure • Aspiration • Secretions • Drug-induced, e.g. ACE inhibitors •  Pharyngeal candidiasis • Treatment-related, e.g. total body irradiation

Lymphadenopathy Lung cancer ± superior vena cava obstruction Chest wall pain Secondaries Pleural effusion

Diaphragmatic weakness, Other phrenic nerve palsy or Anaemia pressure from ascites Uraemia Anxiety Surgery (lobectomy/pneumonectomy) Radiotherapy/chemotherapy (pneumonitis/fibrosis)

Respiratory muscle weakness e.g. MND Fibrosis Lymphangitis carcinomatosa COPD/asthma Heart failure Arrhthmia Pericardial effusion Pulmonary embolism Collapse/consolidation Pneumonia Mesothelioma

Figure28.4  Causes of breathlessness Reproduced from: Lynch J, Simon C (2007) Respiratory Problems, with permission from Oxford University Press.

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Haematological and vascular problems Bleeding/haemorrhage  In all patients likely to bleed (e.g. in end-stage leukaemia) pre-warn carers and give them a strategy.

Severe, life-threatening bleed  Make a decision whether the cause of the bleed is treatable or a terminal event. This is best done in advance but bleeding cannot always be predicted. • Severe bleed—active treatment—b p.1075 • Severe bleed—no active treatment: • Stay with the patient • Give sedative medication, e.g. midazolam 20–40mg sc/IV or lorazepam 1–2mg s/ling. If in pain, consider scopioid • Support carers, as big bleeds are extremely distressing Non-life-threatening bleed:first aid measures • In all cases:reassure; monitor frequently • Surface bleeding—pressure on wound; if pressure is not working, try Kaltostat® or adrenaline (1mg/mL or 1:1,000) on a gauzepad • Nosebleeds—nasal packing or cautery Non-life-threatening bleed:follow-up treatment  Follow-up is directed at cause if appropriate: • Anticoagulants—checkINR • Treat infection that might exacerbate ableed • Consider d bleeding tendency with tranexamic acid 500mgqds • Upper GI bleeding—stop NSAIDs, start PPI in double standard dose and consider referral for gastroscopy • Lower GI bleeding—consider rectal steroids to d inflammation or oral tranexamic acid 9 referral for colonoscopy • Radiotherapy—consider referral if haemoptysis, cutaneous bleeding, or haematuria • Referral for chemotherapy or palliative surgery, e.g. cautery, are also options

Anaemia  Do not check for anaemia if no intention to transfuse. • If Hb 1 rescuer is present, continue CPR whilst this is done. (Some AEDs automatically switch on when the AED lid is opened) • Place one AED pad to the right of the sternum below the clavicle • Place the other pad in the mid-axillary line with its long axis vertical • Follow the voice/visual prompts. Ensure nobody touches the victim whilst the AED is analysing the rhythm If a shock is indicated  Ensure that nobody touches the victim. Push the shock button as directed (fully automatic AEDs deliver the shock automatically). Immediately resume CPR and continue to follow the prompts. If no shock is indicated  Immediately resume CPR and continue to follow the prompts. Use of AEDs in children  b p.1062

Adult basic life support

When to go for assistance  It is vital for rescuers to get assistance as quickly as possible. If you are the only rescuer, go for assistance before starting CPR. When >1 rescuer is available • One should start resuscitation while another rescuer goes for assistance  • Another should take over CPR every 2min to prevent fatigue. Ensure minimum of delay during changeover of rescuers

Duration of resuscitation  Continue resuscitationuntil: • Further qualified help arrives from the emergency medical services • The victim starts breathing normally, and/or • You become exhausted Pad position for external defibrillators Place 1 pad to the right of the sternum below the clavicle. Place the other pad vertically in the mid-axillary line approximately level with the V6 ECG electrode position or female breast (although clear of any breast tissue). Advising about CPR over the telephone  If you receive a phone

call for advice about an adult who has collapsed and is not breathing, call for emergency ambulance support. Advise the person with the patient to perform compression-only CPR. This is easier to describe, more acceptable to the general public, and more effective than traditional compression/ventilation CPR. 0 Children need compressions and ventilation.

Management after successful treatment of cardiac arrest  • Turn into the recovery position (b p.1071) • Give oxygen aiming to keep oxygen saturation at94–98% • Transfer to hospital as soon as possible by emergency ambulance

Further information  Resuscitation Council (UK) Resuscitation guidelines (2010) M www.resus.org.uk

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Adult advanced life support See Figure 29.3. Advanced life support has 3 basic stages: • Revive the patient using basic life support (b p.1054). Basic life support should be started if there is any delay in obtaining a defibrillator but must not delay shock delivery • Restore spontaneous cardiac output, using an automated external defibrillator (b p.1056) or manual defibrillator  • Review possible causes for cardiac arrest and take action asneeded Precordial thump Appropriate only if the arrest is witnessed and a defibrillator is not to hand—may dislodge a pulmonary embolus or ‘jerk’ the heart back into sinus rhythm. Use the ulnar edge of a tightly clenched fist, and deliver a sharp impact to the lower half of the sternum from a height of 720cm then immediately retract thefist.

VF/VTarrest • Attempt defibrillation (1 shock 150–200J biphasic or 360J monophasic) • Immediately resume chest compressions (30:2) without reassessing rhythm or feeling for the pulse. Continue CPR for 2min then pause briefly to check the monitor • If VT/VF persists give a 2nd shock (150–360J biphasic or 360J monophasic); continue CPR for 2min then pause briefly to check the monitor • If VT/VF persists give a 3rd shock (150–360J biphasic or 360J monophasic), then continue CPR for2min • After the 3rd shock (and whilst continuing CPR) administer adrenaline (epinephrine) 1mg IV and amiodarone 300mg IV (lidocaine 1mg/kg is an alternative if amiodarone is not available). Give drugs IO if IV access cannot be obtained  • Repeat 2min CPR, rhythm check, defibrillation sequence while VT/VF persists  • Give adrenaline (epinephrine) 1mg IV immediately before alternate shocks (i.e. approximately every 3–5min)

Non-VT/VFarrest • Start CPR 30:2. Without stopping CPR, check that the leads are attached correctly • Give adrenaline (epinephrine) 1mg IV as soon as IV access is achieved  • Continue CPR 30:2 until the airway is secured, then continue chest compression without pausing during ventilation  • Recheck the rhythm after 2min and proceed accordingly—if VT/VF change to VF/VT arrest (shockable rhythm) algorithm • Give adrenaline (epinephrine) 1mg IV every 3–5min (alternate loops) Fine VF  Fine VF difficult to distinguish from asystole is very unlikely to be shocked successfully into a perfusing rhythm. Continuing good-quality CPR may improve the amplitude and frequency of the VF and improve the chance of successful defibrillation to a perfusing rhythm. Organized electrical activity  If organized electrical activity is seen during the brief pause in compressions, check for apulse. • If a pulse is present, start post-resuscitation care (b p.1057) • If no pulse, continue CPR and follow the non-shockable algorithm

Adult advanced life support

Unresponsive? Not breathing or only occasional gasps Call for help CPR 30::2 Attach defibrillator/monitor Minimize interruptions

Assess rhythm Shockable (VF/pulseless VT)

1 shock

Return of spontaneous circulation

Immediately resume CPR for 2min Minimize interruptions

Immediate post cardiac arrest treatment (b p.1057)

During CPR • Ensure high-quality CPR: rate depth, recoil • Plan actions before interrupting CPR • Give oxygen • If available, consider advanced airway ± capnography—give continuous chest compressions once advanced airway in place • Obtain IV/IO access • Give adrenaline every 3–5min • Correct reversible causes

• • • •

Non-shockable (Pulseless electrical activity/asystole)

Immediately resume CPR for 2min Minimize interruptions

Reversible causes Hypoxia Hypovolaemia Hypo-/hyperkalaemia/metabolic Hypothermia

• Thrombosis—coronary or pulmonary • Tamponade—cardiac • Toxins • Tension pneumothorax

Figure29.3  Adult advanced life support algorithm Reproduced with permission from Resuscitation guidelines (2010) M www.resus.org.uk

Further information  Resuscitation Council (UK) Resuscitation guidelines (2010) M www.resus.org.uk

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Paediatric basic life support Basic paediatric life support (see Figure 29.4) is a holding operation until help arrives. 1. Danger Ensure safety of rescuer and patient 2. Response Check the child for any response • Is heAlert? • Does he respond to Vocal stimuli? • Does he respond to Painful stimuli (pinch lower part of nasal septum)? • Is he Unconscious? If he responds by answering or moving  Do not move the child unless in danger. Get help. Reassess regularly. If he does not respond  Shout for help. Assess airway. 3. Airway Open the airway. Do not move the child from the position in which you found him unless you haveto: • Gently tilt the head back—with your hand on the child’s forehead • Lift the chin—with your fingertips under the point of the child’schin If unsuccessful • Try jaw thrust—place the first 2 fingers of each hand behind each side of the child’s jaw bone, and push the jaw forward • Try lifting the chin or jaw thrust after carefully turning the child onto hisback

• Avoid head tilt as much as possible if trauma to the neck is suspected 4. Breathing Look, listen, and feel for breathing (maximum10s) If breathing normally  Turn the child carefully into the recovery position (b p.1071) if unconscious, and check for continued breathing. If not breathing  or not breathing normally: • Carefully turn the child onto his back and remove any obvious airway obstruction • Give 5 initial rescue breaths—note any gag or cough response Technique for rescue breaths • Ensure head tilt (neutral position for children 150d) despite intense therapy • Type 2 Sudden severe attacks on a background of apparently well-controlledasthma

Management  See Figure29.14, b p.1094 Admit to hospitalif • Life-threatening features • Features of acute severe asthma present after initial treatment • Previous near-fatalasthma Lower threshold for admissionif • Afternoon or eveningattack • Recent nocturnal symptoms or hospital admission • Previous severe attacks • Patient unable to assess own condition • Concern over social circ*mstances If admitting the patient to hospital • Stay with the patient until the ambulance arrives • Send written assessment and referral details to the hospital • Give B2 bronchodilator via an oxygen-driven nebulizer in the ambulance

Follow-up after treatment or discharge from hospital • GP review within48h • Monitor symptoms andPEFR • Check inhaler technique • Written asthma actionplan • Modify treatment according to guidelines for chronic persistentasthma • Address potentially preventable contributors to admission

Further information BTS/SIGN British guideline on the management of asthma (2011 with 2012 update) M www.sign.ac.uk

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Moderate asthma

Acute severe asthma

Life-threatening asthma

INITIAL ASSESSMENT PEFR >50% to 75% best or predicted

PEFR 33–50% best or predicted

PEFR 18y) • Nephew or niece(>18y) • Non-relative living with patient for≥5y The applicant (AMHP or nearest relative) must have seen the patient

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